***March 5, 2016***
Well, it has been a long while since an update, sorry.
I’m happy to start this blog out saying, the kids are doing well.
They are even showing improvement with some things.
Cody and Kayla have now been in the trial at the NIH for over two years. Yes, we all still travel to Bethesda, MD once a month. There have been many benefits of the kids being the trial.
They are getting first class treatment each month at their checkups and treatments. We have learned and been told so much about NPC. This has helped get Cody and Kayla assistances, therapy and help to make their life easier. They are both doing Physical Therapy once a week. They have shown improvement in strength in a few areas. We’ve adapted to a daily life style that is “our life style”. We don’t know everything we need to, or what to expect tomorrow.
The trial is going great. There has been positive updates about Cyclodextrin.
Cody and Kayla are now receiving 900mg of Cyclodextrin. We never thought they’d make it this dose, after their rough start at 300mg. They have both lost a significant amount of their hearing. They are now both wearing hearing aids.
Cody is 20 yrs old, he was 18 when he started in the trial. Six months (July 2014) into the trial, a swallowing test showed he was starting aspirate when he swallowed liquids. Six months later (January 2015), the swallowing test showed that he had improved and was no longer aspirating. He has become stronger in his core, and his balance has improved. He is still sharp with his intelligence. We have even seen improvement with his fine motor skills. He is able, once again, to pour a drink into a glass. Before, his hands shook too much. He is still VERY sociable and loves talking with everyone. His speech is still somewhat slurred, but in all accounts recognizable. Cody is still going to his Metal show concerts that he loves!
Kayla is SWEET 16 now! She was 14 yrs old when she started the trial. The best news I want to report on Kayla is that she has NOT had a seizure in over one year!!!! The seizure medicines she is on now do not cause her daytime drowsiness. She is back to our Kayla that’s full of life. She has also become stronger in her core, and improved with her balance. She has the best support at her high school. She loves going to school and is excited about learning. Her favorite class is Biology. She comes home and sometimes repeats the experiment they did in class.
Some more good news around the corner! The pharmaceutical company is setting up additional locations to administer the drug for the kids in the trial. They are currently talking to Kayla’s Neurologist is Austin. He has agreed to treat Cody and Kayla. It will take some time to set this up. They want all the kids to start receiving the drug every two weeks. This would be (almost) impossible for families to fly to the NIH every two weeks. So, once we get set up in Austin, the kids will go every two weeks for their treatments.
They are very tried/fatigue for 2-3 days after their treatments. They always experience high pitch ringing in their hearing. This is because the drug is attacking their hearing. The hearing aids help some to drown out the ringing. Cody and Kayla said, they basically have just gotten used to the ringing. The kids have started doing their LP procedure awake, instead of being put to sleep. They have a couple of benefits when they do it awake. No IV!!!!! They were both tired of having to get an IV in their arm, and Kayla’s vein were not being cooperative. Doing the procedure awake also shortens their trip by one day.
We have so much in our lives that we are thankful for every day. We have our kids with us. Our kids have the opportunity to be in the trial. We are living in Texas surrounded by family. We live in a community that supports our family.
***January 22, 2017***
Happy New Year! I am past due for an update. I guess I can say, “No news, is good news.”
First, let me update on some personal and fun events
We had a very fun summer going to the beach, concerts, and hanging out with family. Kayla is a junior this year in school…..one more year. This past October, her junior class voted her “class favorite”. She wore a beautiful formal dress, and walk out onto the football field, with her escort, along with the Homecoming court. She was so excited, and this momma was so proud. I was proud of Kayla, but I was just as proud of her classmates for voting for her. Kayla misses so much school. It has been hard for her keep close friendships. To know that her peers remember her, and wanted this honor for her, shows how wonderful those students are at her school. Kayla is always ready to go back to school after she has been out for a week following her treatment.
In December, Cody and a couple of his friends put together a concert with several bands. They turned the event into a benefit for Cody. Cody designed t-shirts, we had them printed, and sold them. Cody just had a birthday and he is now 21! He celebrated his birthday for over a week. No problem, he deserved it. He told me this birthday meant so much to him. I asked him, “why”? He said, that after he was diagnosed he knew most kids with NPC didn’t live to 21 years old, but he beat those odds. I told Cody, he has many, many more birthdays to come. Cody always has so much ambition and dedication for ideas he wants to pursue. Currently, he wants to start his own clothing line.
Now for the trial and treatment: This January 2017, marked 36 consecutive months, flying to the NIH for Cody and Kayla’s treatment. Cody and Kayla have done well over the past three years. Yes, we have seen a slight decline in some areas, but without this trial drug (treatment), that slight decline could be worse. They have both been doing physical therapy. I can see their balance is stronger when they are able to go to PT regularly. This past visit to the NIH, Cody (for some reason), decided to run down the hall back to his hospital room. Dr. Porter and Nicole stood frozen, first because they thought he was going to fall, and then amazed. Dr. Porter said, he was shocked to see Cody run with such force. Porter said, he didn’t think Cody would still be able to run at all. Many friends tell me how good Cody and Kayla seem to be going. Some tell me that Cody appears to be doing better than a year or so ago. We love to hear this.
The trial has started phase 2, and more kids are enrolled, nationwide. The trial is now in the hands of a pharmaceutical company. The pharmaceutical company is opening sites/locations closer to the patients’ homes where the kids can go get the trial drug. So, last March 2016, I gave the pharmaceutical company the name of Kayla’s neurologist at Dell’s Children hospital in Austin. They contacted the neurologist asking him to help set Dell’s Children as a site for the trial. He was on board to help, but stayed very busy, and the set up got slowed down. Well, almost 1 year later, Dell’s Children Hospital is set up for Cody and Kayla to start getting their infusion in Austin, TX. on February 3rd. Best part, Dell is only 45 MINUTES from our house!!! Cody and Kayla can come home after their infusion, sleep in their bed that night and will not have to hang at an airport and airplane the next day.
Cody and Kayla will now be in phase 2/c of the trial. In phase 2, you receive the drug every two weeks, instead of once a month. Scott and I have some concerns about them getting the drug every 2 weeks. All kids in phase 1, including Cody and Kayla, had hearing loss from the drug, and are all wearing hearing aids now. Kayla has continued to have small amounts of hearing loss after each treatment. Cody’s hearing has stayed stabled for several months. So, if Kayla starts getting the drug every 2 weeks now, how much more hearing will she lose? The other concern, the drug causes the kids to be very tired for a few days following their treatment. Kayla, again, is more sensitive to the drug, and it takes her 7-9 days to “bounce back”. When they are tired and their bodies are fatigued, it causes them to be off balance, trouble walking and raises their chances of falling. Kayla uses a walker for the week following her treatment.
We know God will walk beside us and the doctors as we make this transition on February 3rd. We always ask for everyone’s prayers, and support.
We have met all the doctors at Dell and are very pleased. Cody and Kayla were so glad when we got home from our last trip from the NIH. Scott and I are welcoming this new location/transition, as well.
We will take Cody and Kayla once a year (January) to the NIH for evaluation, and testing. They will always stay in the Natural Study for NPC at the NIH.
***October 4, 2017***
Cody and Kayla have been getting their treatment in Texas for 8 months now.
We have made the adjustment from the NIH in Maryland, to Dell’s Children Hospital in Austin easily.
We all enjoy the one day trip for treatment, and no airports. The team at Dell is wonderful, so this made the transition better for us.
Update on Cody and Kayla: the disease is still progressing slowly. The main concerns are their walking and ability to keep their balance. We notice decline in their gait. Kayla walks more with a walker or holding on to somebody, and wears leg braces (AFOs). Cody has trouble standing up without something or someone to hold on to till he has his balance.
There is still some struggle with short term memory loss, speech and swallowing. These have not affected Kayla as much as Cody.
Cody started wearing glasses. I don’t think this is NPC related. He looks very handsome in his glasses.
Kayla continues to have hearing loss. NPC can cause lost to the hearing, but her hearing is being affected more from the Cyclodextrin drug. At this point her hearing aids are helping her hearing ability.
Kayla is a big senior this year in school…. her last year.
We continue to go and have fun, make memories, and live life.
We can not and will not let Cody and Kayla’s life revolve solely around, hospitals, doctors, physical therapy and appointments.
Our kids love life. They want to be involve in all life’s events, concerts, celebrations, vacations. Yes, it is more of a challenge now on them and us to go some places, but the reward is priceless.
Our family continues to be blessed with close family and friends.
We all see and read about so much bad and hate going on in the world. I can say, I am witness that humanity and love is still out there. I see it in strangers that see my kids struggling to walk so they run to open the door for them. They see me trying to get a wheelchair over a curb, and they come and lift the front of the chair. A manager at a restaurant offered to “stand guard” at the men’s restroom door, so I could assist Cody in the restroom.
The Niemann Pick community has recently lost 3 more loved ones to this cruel disease, within 2 weeks. A 5 year old girl, a 20 year old young man, and just yesterday a sweet 30 year old lady from Houston. NPC has no regards for age.
NPC can smother out all your hope with reality.
There are days where Scott and I feel it’s not worth getting out of bed. There are days we have lumps in our throats as we watch our kids struggle to walk and balance. There are days we stop in mid breath as our kids choke on something they are drinking. There are days we fight back the tears because we feel our family has been cheated in life. There are days we feel defeated. We keep fighting and going forward because the one thing that NPC can never take from our family is FAITH!