Graham's Birthday - October 2

Graham's 5th birthday came without him on October 2nd. Matt and I decided to have a few friends and family over to remember Graham together. We wanted to watch videos and tell stories and reminisce about our sweet boy. We figured people would want to acknowledge his birthday and perhaps not know how so, we told them! Everyone brought a gift that Graham would have liked and we took them to the Children's hospital at UNC. Then on Graham's actual birthday we took the kids to the zoo. A place we took Graham. A time to remember and a time to distract ourselves!

These are the words I shared at the birthday celebration:

I had many nicknames for Graham. And more than once a day I would go through my list and he would shake his head yes or no as to which one I could call him. “Graham, are you my sugar lump? My pumpkin pie? My muffin top? My stinky dink? My pookie doo? My snooki snook?”

I miss him terribly. I miss holding him. I miss his smile. I miss watching him dance and reading him books. I miss being constantly in awe of him for how smart and how brave he was.

Graham endured much suffering during his short life. As I attempt to process all that has happened I play each scene over and over in my head and wonder how differently things might have been if this had happened or that hadn’t happened. But I always manage to come full circle, back to God’s sovereignty. If God had wanted things to turn out differently for Graham then He would have orchestrated that. We could have lost Graham many times during that awful 6 month stay at Duke Hospital. But we were blessed with two more years with him. Two years to make precious memories with his siblings and to get a genetic diagnosis that gave us some needed answers.

Thankfully, most days, Matt and I are both able to accept what has happened and continue to trust in the Lord for strength to carry on. I am so thankful for that and I attribute that to the many prayers of our friends and family.

I heard another bereaved parent say that he felt like the paralytic in the gospels whose friends carried him to Jesus on a mat and lowered him through the roof to be healed. When we have been so paralyzed by trauma and now grief, our friends and family have picked us up and carried us to Jesus. I know many of you have probably prayed harder for Graham and our family than I myself have. I’m so grateful. Thank you for continuing to carry us to Jesus.

It brings me great joy when Ben or Hallie mention Graham. They seem to accept the idea that “Graham is in heaven with Jesus.” I don’t think they understand it but they have an answer, and they can repeat it. Occasionally they mention him, and Matt and I talk about him too. Mostly when we doing something Graham liked to do or playing with a toy that Graham liked. A few weeks after Graham passed we were swinging outside and Ben said, “Mommy, I wish Graham could come back home.” He was thinking of Graham because he loved to swing and now his swing was empty.

Just this week while riding in the car, the Hokey Pokey came on. Hallie said “Mommy, Graham really liked this song. He liked to dance to this song at the library at story time.” I said, “Yes, he did, baby.” Then she said, “When Graham comes home we can play this song and dance with him.” As my eyes filled with tears, I said, “Baby, Graham isn’t going to come back home. He’s going to stay in heaven forever. But one day, we will get to heaven to be with him. And then we can all dance together. How’s that sound?” “Ok.” Then Ben chimed in with “But first we have to get baptized so we can go to heaven.” They are excited about baptism since their cousin, Hayes, got baptized this summer. I assured them that you only have to be believe in Jesus to go to heaven. But it’s good to get baptized to show others that you do believe in Jesus. “Ok.”

Thinking about seeing Graham again one day in heaven gives Matt and me great hope. In fact, I often pray, begging Jesus to come back soon and end the pain and suffering of this present world. It seems the veil between heaven and earth gets a little thinner once you have somebody so dear on the other side. Don’t get me wrong, it still hurts like hell even though we know Graham is in heaven. But I read these sweet words recently, that when we see him again, Graham will thank us for enduring the loss so that he could have his reward sooner.

A letter to Graham from Daddy

In the last days of Graham's life Matt somehow had the strength and presence of mind to write this letter to Graham. Matt's brother read it at the funeral. It's been 4 months since Graham died. We miss him so much. I hope to write more in the coming days as we continue to grieve and process all that has happened. 

(This is the portrait that a friend made for us for the funeral and now it hangs in our living room and I stare at it every day! We miss that sweet face.)

This letter is by far the most difficult I have ever had to pen.  No one ever expects to lose a child, and even though we have known for some time that this day would most likely come, nothing can ever fully prepare you for that day.

Graham was the bravest, strongest and most intelligent 4-year-old that I have ever known.  Considering all that he has been through: heart surgery, neck surgery, surviving ECMO, trach placement, hospital stays that number over 8 months total, and the constant needle sticks, trach changes and everything else associated with caring for someone who was as medically-involved as him.  I have never seen nor known someone go through all that he has (or even close to it) and still remain joyful about simple things in life.  Most people would be highly depressed simply based on the surgeries and hospital stays alone.  Yet Graham endured all of this and accepted his life and found ways to enjoy it and not be limited by his challenges and difficulties.  None of this even considers the fact that his vocalization was that of a one-year-old.  He could enunciate about seven words: mommy, daddy, Ben, Hallie, I love you, bye-bye and night-night; and most of these you would really only understand if you had spent a considerable amount of time with him.

These words, however, pale in comparison to his actual vocabulary.  His ability to spell and use his “talker” proved that he had the capacity of someone much older than 4-years.  He could spell his full name, family names, all of his cousins’ names, at least 20 different animals and probably 20 or so other nouns and phrases.  He knew his alphabet, lower case and upper case, numbers, and of course, all of his colors.  You could put a stack of 15 or 20 books from a series in front of him, and he could tell you right away exactly which book was missing.  The fun part of that game was guessing which book you couldn’t find.

Graham also had severe skeletal dysplasia including significant scoliosis.  He didn’t sit up on his own until about 1-year and didn’t walk on his own until about 1 1/2-years.  He never really crawled, but rather sat down and used his feet and legs to scoot along the floor.  Thus my nickname “scooter” for him for quite some time.  Graham never had the ability to run or jump.  He never got to truly jump on a bed, or wrestle with daddy, or get tossed in the air, or learn to kick a ball or many other physical activities.  But this never stopped him from reaching out and grabbing one of the twins by their neck for a big ol’ hug when they were least expecting it.  Sometimes this meant that Graham accidentally “tackled” them when his intentions were to love on them.  The sweetest memories of these three were definitely when Graham felt an urge to hug them, then reached out and the twins decided it was easier to just hug Graham instead of getting a choke hold or tackled.

Graham also loved music and dancing.  Some of his favorite things to do were dance along and do hand motions to many different kids’ songs.  Many days were spent in his bed (during his supposed nap time) watching kids’ music videos and dancing along instead of resting.  I guess he thought it was more important to have a good time, listen to music and enjoy life than take a nap.  Sometimes it was hard to blame him.

Graham loved his toy lions.  He has had many different lions in his life including stuffed lions, lion stickers and a lion costume, but none of them have come close to being his favorite as the two plastic lion figures he slept with every night.  A good friend pointed out one time how fitting it is that Graham would choose a lion as his favorite toy.  She said this was proof that Graham knew who the real Lion was, the Lord Jesus, and that Graham would look to the Lion for security and protection, just as he does to his toy lions.  The Lord Jesus Christ is Graham’s true Lion, and He will always be Graham’s security and protection.

Even though Graham’s life was physically limited and quite short, his impact on others is significant.  I will never know, this side of heaven, all of the people that prayed for him and for us.  Many people who prayed for Graham probably did not know us, his parents or immediate family, but they knew of Graham, his strength, his fight, his battles and his character.  His life was short, but his impact was great.  Graham taught me patience.  He taught me endurance.  He taught me that whatever I had going on in my life separate from taking care of him, it was small potatoes compared to what he had endured.  If Graham could have joy, then I have no excuse to not have it either.

These past few years have been a tough road for Liz and me.  The last several months in particular have mostly been survival.  Many things in life are put on hold when you experience such as we have.  But I have told Liz many times, I would not trade it.  Having Graham has been a joy and the Lord has taught me many things through him and in taking care of him.  Do I wish he were still here?  Absolutely.  Do I wish he had been healed?  With all of my heart.  But I know the Lord has had great plans for our lives, and He is still unveiling those plans through this.  I have full confidence in knowing that Graham is with Jesus; he doesn’t need a ventilator; he doesn’t have trouble breathing; he doesn’t need his talker; he can talk and sing and dance with Jesus.

If you know anything about Graham, you know how much of a momma’s boy he is.  The hardest thing I have ever heard in my life was when Liz told me some months ago: when Graham dies and goes to be with Jesus, he won’t need his mommy anymore.  It was the most piercing thing I have ever heard.  It was scary to think that Graham would be somewhere that his mommy was not.  But on the other hand, he will be with Jesus and he won’t know what it’s like to miss someone or miss something.  He will have Jesus and that is all he will need.

We miss you Graham, but it’s okay, you don’t have to miss us because you have Jesus and we will see you soon.

Love,

Daddy

Breathing Easy

Graham went to be with the Lord on Wednesday afternoon. His kidneys finally gave out on him. He went peacefully in my arms, surrounded by family and friends. He is completely healed and breathing easy in heaven. We appreciate your continued prayers in the days to come for our family.

Graham's funeral will be at Providence Baptist Church in Raleigh on Saturday at 1pm, with visitation following. The burial will be at Hillcrest Cemetery in Colerain, NC on Sunday at 3pm. We are using City of Oaks Funeral Home.

Stage 5 Kidney Failure

It's time to update everyone on Graham's health and treatment. We asked publicly for prayer a while back in making some decisions about his treatment and so I want to share what we have decided. We found out at the end of the summer that Graham's kidney failure was progressing much faster than expected. We were told he had a couple of years before we would be discussing dialysis or transplant and instead we had a few months. 

Graham's nephrologist contacted the kidney transplant team at UNC who quickly turned Graham down for transplant and refused to even meet with us. Since we were unable to get any information from UNC we decided to pursue a second opinion from the Children's Hospital of Philadelphia. They too turned Graham down for evaluation for transplant. The main concern is Graham's tracheostomy and lung issues. To keep a new kidney happy the patient must be fluid heavy for a while which would increase Graham's breathing difficulties. And a person with a transplant remains on immunosupressants their whole life to keep their own immune system from rejecting the foreign organ. Having a trach makes a person more susceptible to infections which wouldn't be good for somebody with a suppressed immune system. It was long, stressful road to find out that Graham would not be eligible for a kidney transplant. 

Once we knew that transplant was not going to be an option we still had to decide whether or not Graham would do dialysis. Because of Graham's small size, heart issues, and other issues he would not be eligible for hemodialysis (the kind most adults do where they go have their blood cleaned several times a week at a dialysis center). But we could do peritoneal dialysis at home. This would involve surgery to put a catheter in his abdomen and putting a solution into his abdomen and emptying it out every night to get rid of toxins that the kidneys are supposed to filter out. The infection risk is very high. The survival rates for children on peritoneal dialysis are not impressive. 80% in the first year. 50% in the second year. Because of Graham's lung issues it was likely that this type of dialysis would cause his lungs to stay too wet and increase his breathing difficulties. Matt and I really struggled with the deciding on dialysis. We knew that our goal for Graham was the least amount of suffering, not necessarily the longest life. In the end his pulmonologist who is also head of the pediatric palliative care team at UNC helped us realize that nothing in Graham's overall health would improve to the point of qualifying for transplant in the next couple of years so its not like if Graham made it through dialysis for a while then there was hope of a recovery. And she let us know that our nephrologist was "going against the grain" to even make dialysis an option for us, but that she offered it because Graham is such a unique case and he was still at home enjoying life. So, after much prayer and much counsel and many tears we decided not to pursue dialysis. We will continue to treat Graham with other medicines to keep him feeling well as possible as long as we can. 

Graham is now in stage 5 kidney failure. We do not know how long we have with Graham but it is likely less than six months. We are begging God for more time and trying to make the most of the time we have. We just got back from a wonderful trip to Christmas Town at Busch Gardens through Make-A-Wish. Please pray for our family as our days are difficult emotionally and physically. Coordinating all of Graham's care is overwhelming. Despite Graham's declining health he continues to learn and enjoy life. He has recently learned his alphabet and how to spell his name! He is such a sweet special boy and we love him so much! 

Summer

Well... I suppose it's about time I post a few updates. I have some health updates about Graham to share soon but I think first I will share what a great summer we had.

We kicked things off by taking Graham to the zoo! He had a good time. He had a hard time seeing some animals that we were far away or not moving or in dark enclosures. But he got to feed giraffes up close that was so cool!

We made a family trip to my parents' house on the Chowan River. This was Graham's first time to my parents since coming home from the hospital with trach/vent/gtube. And this was his second over night trip (we went to the Laneys' at Christmas last year). We had a great time on the water! We got to take a night nurse with us which was really nice.

NC Zoo

July 4th with friends and fireworks

Trip to the River

 

Fun on the deck with pool and water table

Graham's Gtube Feedings

We recently had to change Graham's recipe for his gtube feedings because of the progression of the kidney disease. He needs a diet low in protein, phosphorous, and potassium. The doctors would have preferred to put him on a "renal formula" but Graham doesn't do well on formula. The nutritionist with the kidney clinic has been very helpful in creating a blend of real foods that meets Graham's needs. Though she is inexperienced at creating blenderized diets, she is willing, and she does have the nutritional knowledge that I lack. I mean, what is phosphorous, anyway?

-- Note -- If you don't have a child with a feeding tube then this will likely bore you.

A Little Background:
Graham got his feeding tube placed in December 2014, in the middle of his 6 month hospital stay at Duke. He needed to grow for his lungs to get better and at the time he was not allowed to eat by mouth because of the risk of aspiration when we had his bipap on. (He received the trach in February 2016). He was started on a commercial formula (many different kinds) and for the next four months he was miserable. He was in pain. His reflux was out of control. He vomited frequently. He was put on several GI meds to help him tolerate feeds. We were told he had delayed gastric emptying. We were forced to attempt feeding him in his jejunum (jtube - intestines - bypassing the stomach). This meant 3 trips to radiation to place the jtube. We would turn off the feeds and within an hour he was sitting up and ready to play. Restart feeds and he was miserable.

All the while I was begging the doctors to let us give him real food instead of formula. Graham had been eating real food for 2 years and never had problems like this. However, Duke hospital DOES NOT ALLOW REAL FOOD through feeding tubes. I asked if we could create blends at home. No. Could we give him packaged baby food. No. Could the hospital blend food for him. No. Finally I discovered Liquid Hope, a whole foods blended formula, commercially produced by Functional Formularies and FDA approved. After a few weeks of begging, Duke finally let us try it. He was instantly a different child. More energy. Pain gone. No vomiting. Reflux manageable. Thank you, Jesus. A couple weeks later, he was discharged. Real food was a game changer for Graham.

Since Graham was discharged from Duke last year we have switched most of his specialties to UNC. The two main reasons for this are that the pulmonologist at UNC allowed him to switch to the Trilogy vent (Duke would not) and UNC allows and even supports blended diet, even while inpatient!

New Renal Friendly Recipe:
Graham was on Liquid Hope blended with avocado and coconut oil for a year. The Liquid Hope is intended for adults and is too high in protein for children so we had to add some things to make it appropriate for a toddler. Just last week they finally released a new pediatric version called Nourish! However, we still have to add some things for Graham because of his new diet restrictions with the kidney disease. It took us a few attempts to get a recipe that blends well and is the right consistency to use the feeding pump. We are now modifying his bags (for infinity pump) to allow for a thicker blend.

Graham's current recipe:
1/2 packet Nourish
1 cup applesauce
1 cup fruit (peaches, pears, berries)
1/2 cup rice cereal (Beach Nut brand b/c of low potassium)
8 ounces juice (grape or apple)
1 tbsp olive oil
1 tbsp coconut oil

Soap Box:
Tube-fed children deserve real food. In fact they desperately need it to heal and be healthy. Commercial formulas are mostly high fructose corn syrup and fake nutrition. But doctors stick to it because they can track calories and its easy. Please, if you have a loved one that is tube-fed consider a blenderized diet. If your doctors don't support it then find ones who will. I continue to hear from countless families whose children are vomiting and miserable and not growing on formula. Children who are fed via jtube or who have surgeries to prevent vomiting or who are on multiple meds to tolerate formula. Some children have legit GI issues or other special reasons that make formula their only or best option. But many children do much better on real food! The medical field needs to change how tubies are fed but until they do it is up to parents to find a better solution for their children. And you can do it!

I am happy to discuss anything about tube feeding with you. Her are a few resources if you want more info about blenderized diet for tube feeding.

functionalformularies.com
www.foodfortubies.org

Facebook groups:
Blenderized RN
Blenderized Food For Tubies

Ben taking Graham's blood pressure!

How to modify Infinity bags for thick blends.

A Genetic Diagnosis

Last week, a year after finally seeing a geneticist, we got a diagnosis from whole exome sequencing. They basically took blood from Graham, me (mom), and Matt (dad) and looked at all the genes involved. Graham has Mainzer-Saldino Syndrome, skeletal displaysia from abnormalities on gene IFT140. There are only 20 known cases ever. It turns out that Matt and I both have an abnormal copy of gene IFT140 and Graham got both of them. The chances of two people marrying that have an abnormality on the same gene are like 1 in hundreds of thousands. But since we do the chances of our kids having this is 1 in 4. So the fact that we had two healthy babies, Ben and Hallie, before we knew about this is a blessing and a miracle!

So what does this mean for Graham? Well, it means he will have kidney, vision, and bone issues in addition to the heart and lung issues we are already are facing (Tetralogy of Fallot and malacia requiring trach/vent). The condition causes ciliopathy which means the little cilia that move things in the body are abnormal.
Graham started having high blood pressures a few weeks ago and now we know why. The kidneys regulate blood pressure. Graham has chronic kidney disease and is in stage 3 (of 5) kidney failure. For now that means we have to use medicines to keep his blood pressure under control. Eventually, it will mean complete kidney failure which means dialysis or transplant if those are possible for Graham.

This syndrome is associated with Retinitis Pigmentosa which can eventually lead to blindness. Graham already has limited peripheral vision and can't see well in the dark which are symptoms of this condition. The ophthalmologist says there is no treatment. He hasn't been seen by the ophthalmologist recently so we don't know much more than this. We don't know how long before he has further vision loss. He sees fairly well now as he is able to do puzzles and put beads on a string. But he does run into things on the floor or to his side when walking because he can't see them.

We also recently went to an orthopedist for the first time. It turns out Graham has a 40% curve in his lower spine. That is quite impressive for a 3 year old. They want to do a sedated MRI to check for spinal tethering (the spinal cord attached to the spine) which could be corrected by surgery. Or it could just be scoliosis. They want to make a plastic back brace for him to wear. This will be complicated because of his bell shaped torso and his respiratory difficulties. Of course we already knew about the stenosis (narrowing) in his upper spine which he has had surgery for and may need more in the future. The genetic condition causes abnormality in bone structure and so growth is stunted. That's all we know for now.

So, its not good news. It has been difficult to digest. Through all the crazy health issues and surgeries and hospital stays we have always had reason to hope that Graham would get better as he got older and bigger. Well, now we have found out that he isn't getting better or bigger any time soon. He is actually going to get worse.  I type this update while in the hospital for the second time this week, because Graham got a stomach bug which quickly led to dehydration which made his kidneys angry.


It is helpful to have a diagnosis that ties all his health issues together and in some ways it may give his specialists some insight for proper treatment. But there is no cure (yet) for genetic conditions, we just have to treat each issue the best we can.

Please pray for perseverance and strength for Matt and I and our family. Please pray for wisdom for his doctors. Please pray for Graham to have some joys through all the sickness and for his salvation. I pray for Jesus to come back soon. Before Graham's kidneys fail and before the November election!

Here are a couple of recent pics to remind me and you that we do still have some smiles and good days and we thank God for them.