Matters of the Heart: 2016

Friday, December 16, 2016

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!

Tuesday, November 22, 2016

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

Tuesday, May 3, 2016

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.

Thursday, March 24, 2016

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.

Saturday, January 30, 2016

Jesus talks with Graham

Not too long ago a friend's three old child accepted Christ as her Savior. Praise God. This really made me realize that Graham could be getting old enough to understand salvation. Because Graham doesn't speak it is often difficult to know how much he knows and understands. And I have found that I often underestimate him!

So, a fire was lit in my soul to start being more intentional about talking about the gospel with Graham. I mean we have always talked about God and read Bible stories and prayed but that's not the same as teaching the gospel. That we are sinful and that separates us from God and Jesus came and died for us so we can have a relationship with God and spend eternity with Him. Graham's complex health issues make me even more eager to make sure he understands the gospel since we have no idea what the future holds for Graham or when his time on earth may end. What a weighty responsibility! And what a privilege! Graham rarely goes to Sunday School or preschool or anywhere else that will teach him about the Lord. It is up to Matt and I and I am so thankful that the Holy Spirit has burdened me with an urgency to share Christ with my son. I pray you will share my urgency to share Christ with your children.

For “everyone who calls on the name of the Lord will be saved.” How then will they call on him in whom they have not believed? And how are they to believe in him of whom they have never heard. And how are they to hear without someone preaching? Romans 10:13-14

Graham has been difficult to get to sleep at night for the last couple of months but we have a routine that is working now which is me rocking him to sleep every night. I decided that instead of being annoyed at this extra work I would use the time to talk to Graham about Christ and to pray over him. So every night after we have completed his lengthy bedtime routine I turn out the light, turn on the sound machine, get Graham and glow worn in my lap and talk to him about Christ as we snuggle. I try my best to explain in three year old terms about salvation and heaven and having a relationship with God. I pray God gives me the words and pray God allows Graham to understand.

“You shall therefore lay up these words of mine in your heart and in your soul, and you shall bind them as a sign on your hand, and they shall be as frontlets between your eyes. You shall teach them to your children, talking of them when you are sitting in your house, and when you are walking by the way, and when you lie down, and when you rise. Deuteronomy 11:18-19

Just last week as I was talking to Graham about God, the Holy Spirit guided me to tell Graham that even though he can't talk out loud to mommy and daddy he can talk to God in his head. God knows his thoughts and so he can talk to God without using his lips! He can tell God anything and everything and ask him for help and healing. I almost cried as I was talking to him, realizing as I was speaking what an amazing truth and comfort for Graham this could be if he could understand it. Oh I pray he understands and that God will be an ever-present companion to him!

"Even before there is a word on my tongue, Behold, O LORD, You know it all." Psalm 139:4

It is my desperate prayer that my children become believers at an early age. Please God, remind my soul to take time to teach them about you! If you have experiences or suggestions on teaching toddlers about God I'd love to hear from you!

Tuesday, January 19, 2016

Christmas Review

Oh how I wish I had the time to write of all our joys and struggles and how the Lord provides and sustains. We had a wonderful Christmas, at HOME with our WHOLE family! Last year Graham was in the hospital and we had take out Chinese in the hospital for Christmas dinner. Praise God for allowing us to spend this Christmas together at home.

My (Liz's) parents and brother came and spent Christmas Eve and Christmas Day with us. My mom prepared some delicious meals for us and my brother got a good dose of birth control from being around my three kiddos. We attended Christmas Eve service at our church for the first time and enjoyed that.

After Christmas we traveled to Matt's parents for a couple nights. This was our first time traveling with Graham since he has had the ventilator and gtube. We had no nursing while we were there so we were busy and tired. But it was nice to be there with all of Matt's family. Nothing went catastrophically wrong, we didn't forget any important equipment or meds and we had no equipment failures. Graham did get a few impressive booboos from his cousins. But all in all it was mostly a successful trip.