Ryley’s LCH & Diabetes Insipidus Ongoing Journey
Ryley was rushed to Comanche County Memorial Hospital by ambulance in the early morning hours of May 3rd after having a seizure due to low sodium. The diabetes insipidus cause by the Langerhans Cell Histiocytosis (brain cancer) was the cause of he low sodium event. He spent 2 weeks at CCMH in Lawton. They were unable to get the sodium stabilized. Then he was transferred to Cincinnati Children’s Hospital on May 15th by air ambulance. After about 10 days the medical team at Cincinnati were able to get the sodium stabilized.
The seizure and low sodium reeked havoc on his muscles. He now has problems swallowing and has to have liquids thickened so he does not aspirate. He has a feeding tube in his stomach to help keep his body nourished. Before all of this he could stand on his own and walk with assistance. Now he cannot.
On June 10th he was transferred to The Children’s Place Rehabilitation Hospital in Bethany OK for him to continue his rehab and be closer to home and near family. He started PT/OT/ST and is tolerating it well.
We are asking for help to cover expenses that insurance doesn’t cover, modifications to the bathroom at home so he can roll into walk-in shower and a medical grade machine to check his sodium at home (I-stat).
The I-stat is upwards of $20k which of course insurance will not cover, and bathroom modification is estimated $5-7k.
We thank you in advance for your monetary help and ask that you pray for Ryley.
The background of Ryley’s Illness:
In March of 2009 my son’s second grade teacher ask me if I had noticed that he was going to the bathroom often. I replied yes and we both agreed that she should be seen by his pediatrician. I took him to the doctor and she thought he might have Diabetes Mellitus. She ran a couple of tests and they came back normal, much to our relief. Spring turned into baseball season, which then turned to summer and football practice began. We were still noticing that Ryley was drinking water by the gallons and it was coming out just as fast. But we live in Texas and the outside temps were over a hundred degrees, so we were not that worried. School started back up and within two weeks, Ryley’s third grade teacher was concerned that he was in the bathroom too much. So, I called his doctor and she was on vacation. I didn’t care too much for the on-call doctor, so I made an appointment with a pediatrician that is also a friend of mine. She examined Ryley and she thought maybe Diabetes, but also thought of Diabetes Insipidus. She ran a simple test and it was indicative of Diabetes Insipidus. She talked with a Pediatric Endocrinologist at Cook Childrens Medical Center in Fort Worth, who gave her a list of lab test that need to be performed before she would see Ryley. Our insurance requires the PCP to order any lab test, so we took Ryley to back her. When we got into the exam room, I explained to nurse what the last 5 months had been like (Ryley drinking over 120 ounces of water and urinating over 30 times in 24 hours), and she proceeded to tell me that I was letting him drink too much water and just to cut his water down. She didn’t even want to let us talk to the doctor, but I insisted. The pediatrician came in and I explained that Ryley was getting up approximately 15 times at night to urinate and drink more water. He was drinking water all the time, and I had another doctor check him out. I gave the list of labs the endocrinologist wanted done and asked for a referral. I was expecting her to be concerned and give the referral and order the tests, but she instead, got very mad! She said there is nothing wrong with your son! We argued and she finally ordered the tests and said once the results back, she would determine if a referral was need. A couple of weeks went by and the nurse called stating all the results were normal. I asked if she would fax me the results and she ask why I needed them, that she had already given me the results. I explained that I was going to get a second opinion, and she replied that they would fax them to the second opinion doctor. I told her that I had a right as a mother to see the report and after arguing, she finally faxed it to me. Upon review of the tests that she had ordered, I realized that she had not ordered the one blood test that would tell us if Ryley had Diabetes Insipidus. I was livid!!! So I called our insurance company, Tricare (my husband is retired military), and asked if we could change PCPs. They immediately changed PCPs and I took Ryley to the new doc with all the medical records I could get from the old PCP. (I had to fight tooth and nail just to get some of his records). After examining Ryley, she said his water intake and output might be psychological, but she would order the blood test for Diabetes Insipidus. She said in the meantime, to not let Ryley have water at night, in hopes that he would sleep. The first night was horrible!!! Ryley got up seven times to urinate and I withheld the water. We cried together, as he begged me for water. The next night was the same; however, I crumbled under his tears. I just couldn’t bear him begging for water. So we got the results back of the blood test and it did show that something was wrong.
So we took him to the Pediatric Endocrinologist, who ordered a water deprivation test. This had to be done as an inpatient procedure, because if Ryley had DI, then the test would take him to the brink of death by letting his body dehydrate. The test was devastating for Ryley!!! He was not allowed to eat or drink for 17 hours to see if his kidneys would concentrate his urine. After the 17 hours, the test was inconclusive and then he was sent for an MRI of his brain. And sure enough the “bright spot” for the pituitary gland did not show up on the MRI, so they diagnosed him with DI and started him on DDVAP (synthetic hormone) to help the kidneys concentrate the urine. (This keeps him from being thirsty because the body absorbs the water it takes in). We now are ecstatic that we have a diagnosis and medicine to treat the symptoms. But that was short-lived, because the next sentence out the doctor’s mouth was that DI is often caused by Langerhan’s Cell Histiocytosis. The doctor explained that DI is commonly seen first in LCH patients and that Ryley would need to have an MRI every three months for the next five years to look for lesions caused by LCH.
LCH has typically been thought of as a cancer-like condition. In Ryley's case, his pituitary stalk had become thickened. And now is pituitary gland is shutting down, causing hormone deficiencies. So Ryley would now be also followed by a Pediatric Oncologist to monitor the DI and the possible LCH.
The next 8 months were pretty normal, Ryley was taking the DDVAP and his water intake and output stabilized and the MRIs were coming back normal… Life was good! But then in June of 2010 his MRI of the brain showed lesions. So we talked with oncologist who was not sure how to proceed with treatment. We were told that the oncology team would consult with each other and develop a treatment plan. We waited a few weeks and we heard nothing! So I called the clinic and was told that the oncologist was no longer employed there and we would be assigned a new doctor. But they could not tell me who the doctor would be and it would be two and half months before Ryley could be seen again, because they were now backed up with patients since they were down a doctor. This didn’t set to well with me, so I started researching LCH and LCH doctors on the internet.
I came across Dr. Ken McClain in Houston, and I thought wonderful, he is only four hours away! So, I called his office and made an appointment. The only bad thing is that we had to wait about a month to get in to see Dr. McClain. But this gave me time to gather all the medical records. Dr. McClain then called and talked with me for a while asking if we could get a PET Scan done here before we came to see him. I made some phone calls to the insurance company and Ryley’s new pediatrician and got the scan scheduled along with another MRI. So, on September 13, 2010 we finally saw Dr. McClain, he examined Ryley. He explained that LCH can be a slow progressing disease and manifest in many different ways. He seemed to think Ryley might have neurodegeneration going on, but wanted time to review all the medical records. So we left knowing that Ryley probably did have LCH, but did not know how or if it was treatable. What we did know was that the PET Scan did not show any other lesions in body, just in his brain.
On September 15, 2010, two days after seeing Dr. McClain, we saw the new oncologist in Fort Worth. While sitting in the exam room waiting to see the doctor, Dr. McClain called my cell phone. He had reviewed Ryley’s records and determined that Ryley had Neurodegenerative Central Nervous System Langerhan’s Cell Histiocytosis (ND CNS LCH). And he needed to start treatment. I said alright, what is the treatment… He replies back… low dose chemotherapy. I am stunned!!! Not prepared for this!!! I can’t cry, because Ryley is sitting right next to me. I am able to hold it together to explain where we are at and I would like Dr. McClain to speak with the new oncologist. So the new oncologist comes into the room shortly after I talk with Dr. McClain, and I explain everything that has happened in the last year and a half including the phone call from Dr. McClain. He asks “how did you hear about Dr. McClain”? I said the internet, and then he explains that Dr. McClain is one of the top three doctors in the world for LCH. And he is the number 1 doctor in the United States. So, I felt and I knew that we were in good hands!!!
Ryley has endured over 150 chemotherapy infusions and years of oral targeted chemotherapy.