Happy Rare Disease Day. -- Happy? ? ? Yeah, no.

Rare Disease Day was February 28.  I usually make a post to my social media accounts to spread awareness but this year I missed it.  I saw the other posts scrolling by in the newsfeeds and enjoyed seeing all the beautiful faces of kiddos who are battling life-threatening, rare diseases.

When you have a child with a rare disease or disorder it’s such a very different world.  You want to spread awareness but it’s almost impossible to explain what we go through on a day-to-day basis. The disparity of emotions, trying to keep your child alive, battling insurance companies, fighting for services, trying to find the appropriate education plan, and then just trying to be parents to your other kids and a wife to your husband!

Willy had lissencephaly which is a neuromigrational disorder – not a disease.  But those terms are used interchangeably in the context of Rare Disease Day. Lissencephaly is a disorder in which the brain of a fetus does not develop properly at the end of the first trimester. Whereas a neuro-typical brain develops folds or grooves which gives you motor skills, a lissencephaly brain lacks grooves.  There can be a wide range of groove depth resulting in different variations of lissencephaly.  Willy is even rarer than other lissencephaly cases because he’s not missing the gene that has been linked.

Willy was on the more serious side because his brain was super smooth like a bowling ball.  He also had pockets of water in his brain called hydrocephalus. The result was a Willy with very little (read: almost non-existent) motor skills.  In general, he presented with the cognition and motor skills of a three to four month old. In other words, he was our big baby.

He didn't walk or talk or grasp things in his hands. He lost the ability to suck and swallow so he took his nutrition via a stomach tube. He had daily seizures which caused him to stop breathing. We had a pulmonology routine that took hours each day and night. Trying to keep him suctioned out and his airway clear was a full-time job. (Breathing isn’t option, right?)

He was happy though. He loved to snuggle. He cooed. He watched movies. He listened to music. When my other two kids got “too old” for mom snuggles, Willy was always willing and able. I enjoyed watching him grow through the years and do things we thought he wouldn’t do.  He had wonderful school teachers and therapists who worked with him day in and day out to teach him things.

Willy was on home hospice the last two years of his life. He still went to school and had a good quality of life but we stayed out of the hospital.  We lived a more “natural” life. We still gave him antibiotics if the need arose and we had all his prescriptions and oxygen and breathing treatments but we had decided no more resuscitation or aggressive interventions.

Truth is, the 11 years Willy was here on this planet were some of the best, if not hardest, of my life. It changed my heart. My entire being. I look at life so differently now, especially towards those who are different. I’ve advocated, sat on boards, moderated groups, started a 501c3, testified in congress both in Michigan and on the national level and more. I’ve tried to make some positives out of what could be perceived as a huge negative. (Not Willy, but his disorder.) I have tried to keep up a sunny attitude and am able to do so for the most part.

But face it. Lissencephaly sucks. Rare diseases suck. Rare disorders suck. And rare only means it doesn’t get the funding that cancer and other more prolific disorders and diseases do.  In art, rare is good. In diseases, rare is not. It just means no one will ever know what you’re talking about.  It means you will have to educate many of the professionals, doctors included, who will work with your child. (Yes, I’ve had medical doctors not know anything about lissencephaly.) It means there won’t be much funding or money available for research or assistance.

So Happy Rare Disease Day – late.  

http://www.rarediseaseday.org/

https://rarediseases.org/rare-diseases/lissencephaly/

https://www.ninds.nih.gov/Disorders/All-Disorders/Lissencephaly-Information-Page

Bi-Partisan Action and Pediatric DNR's in an Educational Setting

Thursday, January 26, 2017 I went to Lansing to meet with state Senator Rick Jones along with Dr. Ken Pituch, Willy's hospice doctor and a pediatrician with the University of Michigan, Maureen Giacomazza, a nurse consultant with the University of Michigan Pediatric Palliative Care Program, Debra Chopp, University of Michigan law professor and attorney, and two University of Michigan law students Russell Busch and Dorian Geisler.  Russell and Dorian have been working diligently to prepare a case for us and my hat is off to them.

Our goal for the meeting was for the Senator to hear our appeal to amend Michigan's DNR Procedures Act so that it clearly and fully covers children in school--and then decides to more or less immediately introduce our proposed amendments as a bill into the Michigan legislature.  The bill then passes, and then the law is changed: Parents can get DNR's for their children explicitly by law, and schools need to respect those DNR's explicitly by law.

Background:

There is one statute in Michigan that covers DNR's which was passed over twenty years ago.  It's called the Michigan DNR Procedures Act.  It says nothing about parents' ability to make DNR's for their minor children and it says nothing about schools.  This creates ambiguity and uncertainty for both parents and schools.  

We want to amend the act by adding language that explicitly says that parents can get DNR's on behalf of their minor children and explicitly mentions schools as a context in which DNR's need to be respected.

We're trying to frame this change as a "win-win," a way to clarify the law so everyone is on the same page.  We want bi-partisan support.  We've spoken to Right to Life of Michigan.  Other lobbyists have spoken to the Catholic Coalition.  Almost all the signs we've gotten from people we've talked to have been positive.   

The potential obstacle is school districts and their lawyers--who, understandably, are worried about liability.  The act would cover a huge diversity of schools all over Michigan, both now and (theoretically) twenty, even fifty years from now.  Districts and their lawyers are trying to be careful and anticipate something that might go wrong (and get them sued) in the future.  

It's a win-win because we are also willing to add language to the DNR Procedures Act so that it says explicitly that teachers or staff who act responsibly (and who perform comfort care measures, say) are immunized from liability.  The win-win: parents explicitly get the ability to have DNR's for their kids in schools, schools explicitly get immunity if their personnel are doing the right thing.

Of course, there are still lots of little complex details--about how a student's DNR might fit in with her IEP, for example--but that's the basic idea.  We are willing to work with the Senator and other Michigan legislators so that the language about all the little things is just right.  But that's a concern for after we get the Senator on board.  Our first goal is to make the Senator think that (1) this issue is a good cause and (2) that actually passing these amendments, in some form, is politically doable--that there's enough support.

I was sitting right next to Senator Jones and when it was my turn to present, I turned my chair so I was facing him and read my statement.  For that moment, it was just the two of us in the room and I was telling him about Willy. He listened earnestly and was very compassionate.  When I was done he shared with me that although it wasn’t quite the same, he too had a special needs son.  In that minute, he wasn’t a Republican and I wasn’t a Democrat.  He wasn’t a senator and I wasn’t a constituent.  We were simply two parents sharing about our special needs children.

(Interesting twist: Unbeknownst to us, Scott Menzel, Washtenaw Intermediate School District Superintendent, had just days earlier met with Senator Rebekah Warren and she had started the process of sponsoring a similar bill.)

The end result of the meeting was that he was very happy to either sponsor, co-sponsor with Senator Warren, or help in any way he could. Follow-up emails to his office assured us they had already contacted Senator Warren’s office to discuss assisting.

As the bill goes through the legislative process there will be more meetings, committee hearings, and testimony so I will keep everyone posted.

In a time where our country is more divided than ever, it felt good to sit with Senator Jones and see the wheels of democracy in action. 

http://www.mottchildren.org/profile/1132/kenneth-pituch-md

https://www.law.umich.edu/clinical/pediatricadvocacyclinic/Pages/default.aspx

http://www.senatorrickjones.com/

https://www.legislature.mi.gov/(S(izncxegpu4xl2mqzb3v3ru45))/documents/mcl/pdf/mcl-Act-193-of-1996.pdf

Pediatric DNR's and the Right to an Education: Our Day in Court

I just met with the University of Michigan’s Pediatric Advocacy Clinic student attorneys.  They have been diligently working on the pediatric DNR issue under the direction of U of M law school professor Debra Chopp. 

We have all been working on this issue for about 2 ½ years now; trying different ways to “force” all the school systems in Michigan to honor pediatric DNR’s. The processes are sluggish and it takes a lot of patience to watch it all roll out.  Our prior attempts came to a grinding halt when Willy passed because he was the plaintiff.

This time, we are going straight to the Michigan Legislature. Senator Rick Jones (R-24^th Michigan Senate District), who over the years has been a friend to the Pediatric Advocacy Clinic, has agreed to hold a testimony day next Thursday. He is sympathetic to our cause and is interested in learning more.

If it goes our way, he will agree to sponsor a bill to amend the language of the Michigan Do-Not-Resuscitate Procedure Act of 1996 so that schools will have the required protection from liability that their lawyers have wanted. In essence, they will be “forced” to honor DNRs in the educational setting. Considering who Senator Jones is and the fact that we have a Republican majority in both the house and the Senate AND a Republican governor, chances are great that the bill would be passed. (It goes without saying that most Democrats would support this as well.)

Senator Jones will not support any bill that doesn’t have the support of Right to Life.  Right to Life and the Archdiocese of Detroit both have to either support the bill or at the very least be neutral. The law students did their work, reached out to both groups, and received their support. This makes him more interested and brings us closer to our goal.

Next Thursday I will be meeting with Senator Jones along with Debra Chopp, Dr. Ken Pituch from the University of Michigan Hospice and Palliative Care program, and the student lawyers.  It will be closed door testimony.  My hope is that our testimony will be compelling enough for him to agree to sponsor a bill on the spot.  The reality is that the meeting could go many ways. He could say no. He could put it on the back burner.  He could red pen our proposed language change and send it back to us.

This issue affects a small part of our population. It’s not “sexy” legislation. It’s largely bi-partisan. If this bill gets passed, it won’t create world peace of solve homelessness. But to those affected by this issue, it is life and death. It is part of taking care of the most vulnerable of our population, our children who are terminally ill. It will allow us to feel comfortable giving them an education with their peers and know that they will be properly taken care of in school.

I’ve practiced my statement in front of different groups of professionals. I’ve taken their critiques and changed a few things.  I’m ready.  This is the most hopeful I have been about resolving this issue since we started this fight.

If anyone is in Senator Jones’ district (Clinton, Eaton, Shiawassee, and parts of Ingham County – Locke Township, Wheatfield Township, Williamston city, and Williamston Township) please let me know.  I am going to prepare verbiage you can use to send his office a quick email in support of this.  I am not a constituent of his and it doesn’t necessarily matter if he agrees to sponsor the bill.  However, if he has constituents who are in favor, it will help our case if he hears from you.

I’m including below my statement. Please wish us all well. 

My Statement:

First, thank you, Senator Jones, for arranging this time for us to come speak.  I know there are constant demands on your time and we are all very appreciative of this opportunity. I am excited to introduce you to my son, Willy.

My name is Dawn Krause and I am from Saline.  I am married and have three children.  I work as a research administrator at the University of Michigan.  I received two degrees from Michigan State University; one in Eastern European History and one in Interdisciplinary Studies in Social Sciences with a cognate in International Political Science.  Willy’s father works in law enforcement and has a degree in anthropology and his step-mom has degrees in education and social work. My husband has a PhD in American Studies and is a professor at Central Michigan University.  I give you this background for no other reason than to explain how no life experience, job, or level of education could ever prepare you for what we have had to go through.

My middle son, William, (Willy), was born in March of 2004.  My pregnancy with him was normal and uneventful as were his first four months of life. In July of 2004 after a seizure he was diagnosed with lissencephaly.  Lissencephaly is a rare neuromigrational disorder in which the brain does not form correctly during gestation.  Willy had no gyri or ridges which are what gives you your motor skills.  At diagnosis, we were given a two-year life expectancy for Willy.  We were told to take him home, be prepared to deal with complex seizures and respiratory issues and basically try to keep him comfortable while we waited for the end.

The early years were very difficult. We were learning how to take care of this very sick baby and doing everything we could to keep him alive. Every time he was sick we thought it was the end. Mott Children’s Hospital became our second home. The stress of the entire situation was maddening to our family, to our other children, to our jobs, our finances, and our marriage.

After a while we started to pick up some confidence and hope. Willy started to have longer periods of improved health. He was enrolled in Early On in Jackson County and the therapists and teachers there taught us how to give him a quality of life. They opened up a new world for us. A world where special education meant a life for Willy with goals of his own. They had equipment at school that we could have never afforded to have at home. They had specialists there who had studied how to educate and work with this very special population of students. They focused on what he could do not what he couldn’t do.

Sometimes their goals were lofty which made us laugh. We joked that we really just wanted him to poop and breathe. (Constipation was a large part of our lives and caused a lot of problems for Willy.) They wanted more for him. He learned to hold his head up. He learned to make choices with his eyes. He enjoyed swimming in the therapy pool which was not only fun for him but good for his muscles. He received physical, occupational, music, and vision therapies.  They went on outings in town.  They did crafts. They listened to stories. He worked hard during therapy sessions and would come home exhausted. He was healthier when he was in school because it is a much healthier lifestyle than just lying around at home. After years of working with occupational therapy he learned how to hold a toy on his own. He learned how to roll over.

Unfortunately, as I mentioned, lissencephaly is a regressive disorder.  This means Willy’s performance peaked at a very young age and started to decline slowly every day thereafter. Mind you, it was a slow regression and we had a lot of really good years in there. But his disorder, along with severe seizures, pulmonary issues, and cerebral palsy, was slowly destroying his muscles. Any skills he had (swallowing, holding a toy, rolling over, holding his head up,) were slowly going away.   Eventually we had to put a feeding tube in so he could get enough nutrition.  Your muscles control everything; how you eat, swallow, breathe, urinate – everything. His respiratory muscles were especially damaged from chronic pneumonia.

Even so, the educators and therapists working with us just kept changing the goals. Adapting. Meeting him where he was at. He continued to hit goals and milestones and then he’d regress and lose some skill or function.  We’d celebrate the gains, no matter how small, and we’d grieve the losses. For the most part, he continued to be a happy boy, enjoying music, the pool at his school, outings with his class, snuggling with loved ones, and his iPad with his shows.

We had maintained a very pro-active and aggressive approach to his care. Even though he had been given an approximate life expectancy of 2 and he was now 7, his life had been much better than we ever could have anticipated. When the hospice and palliative care teams would come visit our hospital rooms we promptly sent them away. We knew Willy had a lot of life left to live.

Willy’s lung function started to go seriously downhill somewhere around 2009.  We wound up in an on again-off again cycle of hospitalizations that lasted over three years.  Usually he’d start with a cold or sniffle and the next thing you know we were in the intensive care unit and he was fighting for his life.

In late fall of 2012, Willy had been having some increased breathing and swallowing issues due to the continued regression and his obstructive sleep apnea. We had an appointment with Dr. Charles Koopman, an ear, nose, and throat physician, to see what our options were.  The options that were presented to us were not good. All of them required some form of complicated surgery.  The only real viable option for us was a tracheostomy which, on one hand would have made life a little easier, and on another hand would have seriously increased the complication factor. We decided against the trach.

That night when we got home, I couldn’t help but think that it was the “beginning of the end.”  There were no more procedures or life-saving surgeries that were options.  It became a situation where we would just use what we had to keep him comfortable. His regression had made it to that crucial juncture where we realistically couldn’t do much more to keep him alive with the quality of life that he deserved. It was at this point that I realized Willy was terminally ill but not actively dying. This phrase would become the basis for all the decisions we made from then on. We needed to adjust our decision making accordingly.

In June of 2013 Willy was hospitalized for increased seizures. This wasn’t something new. He’d have a growth spurt, or his hormones would change, or a medicine he was one would lose its efficacy. We were always looking for the right concoction of medications that would keep his seizures at bay but not drug him out. I’ll leave out the details but during this hospital stay, it was presented to us once again that we could consider palliative care or hospice services. Not only had we tried almost everything we were willing to try, Willy had gone quite a bit downhill in the years preceding. It was time for us to have the talk about where we go from here and whether or not it was time to consider palliative care or hospice services.

The ACA allows for pediatric hospice patients to also receive specialty physician care in cases like these. These patients require comfort measures that hospice provides and also curative care that comes from specialty clinic visits. This would make the perfect support set-up for the last few years of Willy’s life. If we could have designed perfect care for our imperfect situation, this would be it.

With our goal being the best quality of life possible for the time that Willy had left (remember – he was not actively dying at this point,) we decided to go home from the hospital on hospice services. Willy would still be able to attend school. We would have support by way of having supplies delivered, oxygen in the home, and morphine and other comfort measure drugs available to us for his care.  All of our medications would be ordered by the nurse who came once a week to check on Willy and delivered right to our home. If Willy became ill, a call to hospice would send out the nurse or the doctor. If Willy needed antibiotics we could get them. If Willy took a turn for the worse, we could either provide the comfort measures we had in the home or we could change our minds and head for the hospital for more aggressive treatment. We were also able to still see our physiatrist and our neurologist in clinic for specialty care.

We worked with a team of professionals to design a medical care plan which included a “do not resuscitate” (DNR) order. The decision to add the DNR into Willy’s care plan was no less agonizing just because we knew it was the right thing to do. Taking all of the facts into consideration we knew it was time. Every time Willy was sick he came back to us a different boy; less of his old self and more tired and uncomfortable. We knew that should something happen where his heart stopped beating, we would not want that for him. We would want comfort measures. We would want him to be surrounded by people who love him, whether at home or at school, holding his hands and staying by his side. Once we made this decision we never looked back.

At the time, Willy was attending Haisley Elementary in Ann Arbor, Michigan and they honored his DNR. We had a team meeting to put protocols in place and off to school he went.

The next school year we made the decision to transfer Willy to the Washtenaw Intermediate School District.  They have a specialized special needs program and there were more services available for Willy including a very warm therapy pool which we knew he would love.  We were sad to leave Haisley but, again, keeping in mind quality of life, we knew it was the right decision.

When we found out that the WISD would not honor his DNR we were crushed. We would have been devastated if something had happened at school and resuscitation was attempted. I am very sure this is a difficult concept to understand for parents and adults who do not live in a world where children are terminally ill. But we did live in that world and it was not acceptable that school district lawyers could overrule our decision with regards to our DNR.

We decided to still send Willy even though we knew they would not honor his DNR. We knew the pros outweighed the cons. Willy loved school. He loved the pool. He needed the therapy and the stimulation they offered there. However, we did start a lawsuit against the district to try to force them to honor the DNR.  The suit was still in progress when Willy passed in November of 2015.

For Willy and kids with these regressive disorders, the “end” can be years. In the meantime, they have to live. They deserve an education. They deserve a life of their own with an educational program designed for them. This is a right under the Free Appropriate Public Education (FAPE). FAPE is an educational right of children with disabilities that is guaranteed by the Rehabilitation Act of 1973 and the Individuals with Disabilities Education Act. The fact that they have terminal disorders and may have a DNR should not infringe upon their right to an education. The school districts that serve our state must be consistent in their policies regarding pediatric DNR’s. Their policies should not change across town or across another district’s border.  Just as you’d write a medical care plan that included an epi pen for a student with a deadly allergy, medical care plans for student’s with DNR’s should also be included in the educational setting.

When a parent or guardian reaches the agonizing decision to write a DNR for their child, no one should be able to tell them that the DNR will not be honored. Especially in an educational setting where they have the right to have the same experiences as their peers right up until the day they pass. No child is less entitled to their rights to FAPE because of a diagnosis, prognosis, or a medical order.

I ask you today to consider our story but please know there are many more out there just like ours. I ask that DNR’s be honored in all educational settings in our great state. I ask that the laws allow medical care and educational professionals to team up with the families to write appropriate care plans that are as individualized as the student. Proper protocols and policies will follow so that staff is comfortable and knows what to do in case of a situation with a student. Many districts already honor DNR’s and have put appropriate policies in place. Something as important as this, which really comes down to life and death, should be consistent between every educational jurisdiction in our state. Please work with us to help make it so.

Thank you.