It's All in the Bones

 



Nadiya's hip x-ray with the
hardware still in place after surgery

The Bare Bones of the Matter


        
              When things feel as if they have calmed or that life is becoming balanced and controlled there is always something to jeopardize that balance.  As I looked forward to summer, well aware of the many tasks ahead of me, I still felt Nadiya’s health was good.  Things were going well with Nadiya medically. That is until Nadiya had a Dexa Scan which is a scan to measure bone density. The scan specifically looked at the hips and the spine which are common problem areas. Typically this becomes an issue as we age and particularly for woman. As we age we can develop osteopenia which is a condition where the bone mineral density is lower than normal. This can be a precursor to osteoporosis which is a disease of the bones where the bones become porous and are at high risk of fracture or break. Some causes are lack of weight bearing exercise, inadequate vitamin D and Calcium intake, medications, chemotherapy, among others.  Some of you may recall me talking about this many years ago when Nadiya first saw an orthopedic surgeon.  We always knew Nadiya would be at risk.

Osteopenia and Osteoporosis are unusual in children but Nadiya has several risk factors. Think of yourself as a child or think of your own young children. In the first few years of life you climbed and walked and jumped exploring whatever you could reach. Nadiya spent her first few years unable to sit up and unable to walk. Her weight bearing activity consisted of the time she could tolerate being in a stander. So as Nadiya's bones grew she didn't have the weight bearing activity to strengthen them like typical children do. This is why she had the subluxation in her hips which resulted in surgery to place the hips properly in the sockets and reform the proper angle of the femur bones.  It is common for children with severe Cerebral Palsy to have hip problems and bone mass problems.

In addition to that risk factor Nadiya has been on the Ketogenic diet for 6 and a half years. The diet is significantly controlling her seizures but it is nutritionally deficient. This is why Nadiya has had to take a special Nano Vitamin supplement twice a day. Last year at the request of my dermatologists I asked the Keto Team about increasing Nadiya's vitamin D and Calcium. Keep in mind that much has changed over the period of time that Nadiya has been on this diet. When a child was born with Epilepsy it was thought that medication should be attempted first to control the seizures.  The Ketogenic diet was considered a last resort. Now if a child is diagnosed with Infantile Spasms, which Nadiya was, the diet is recommended as the first course of treatment.  Research has shown that the children with Infantile Spasms have the best results when the diet is started immediately upon diagnosis. Nadiya was two when she started the diet and only after the failed attempt of several medications. Today it would have been tried first. For whatever reason the diet works for Nadiya but only when she is on it. Many children with epilepsy go off the diet and remain seizure free. We know that isn’t the case for Nadiya so she will be on the diet long term. Just recently neurologists are realizing the long term affects of the ketogenic Diet on a child's bone density. This is what prompted Nadiya's Dexa Scan. My dermatologist seemed to be a step ahead as she pondered the affects of the diet years ago after having met Nadiya and seeing her fair skin. That is how a dermatologist thinks.   A neurologist is focused on seizure control which is usually the urgent and most prominent problem.

Another risk factor for Nadiya is one of her anticonvulsants she has taken for almost 4 years which is Depakote or Valproic Acid. It has been shown to increase the risk of Osteoporosis in adults and children. But it works with the Ketogenic diet in keeping Nadiya seizure free.

 

 

 

Make No Bones about It

So my friend Lisa and I took Nadiya to Hopkins for the Dexa scan. Lisa had to hold Nadiya's knees in place while I kept her arms up and away from her chest. It was a very quick scan and Nadiya was extremely interested in the imaging machine that moved back and forth over her.  This made Nadiya easy to manage during the quick scan.

Nadiya's neurologist called me two days later to tell me that Nadiya had Osteopenia in her hips and that her spine was worse and of concern. He did not say she had Osteoporosis for sure in her spine but that we should see an Endocrinologist. Endocrinology is the branch of physiology and medicine concerned with endocrine glands and hormones. They address things from diabetes, thyroid conditions, and problems with reproduction to metabolic disorders. Nadiya's neurologist spoke to a Pediatric Endocrinologist at Hopkins who is familiar with the Ketogenic Diet. Because it is so difficult to get an appointment with a pediatric specialist Nadiya will have to wait until September.  The appointment was made last spring. That has given me a great deal of anxiety but the neurologist feels it is ok to wait until then. He thinks Nadiya will need additional supplements but they will have to be Keto-friendly.

Nadiya walking with Yuba the Therapy Dog

 at Mt. Washington Pediatric Hospital

My guess is that Nadiya will require more weight bearing activity as well and that additional physical therapy will be necessary. Right now Nadiya has weekly PT at school and daily weight baring activity throughout her day. We have not been accessing private PT because our insurance plan now requires a co-pay of $40.00 per session. Imagine if you needed rehab after an injury or a surgery. You might need PT 3-5 times per week. How many people would ignore the order for PT because they couldn't afford it?  I worry so much about Nadiya's future healthcare needs. It is getting worse every year as Nadiya's needs increase and our insurance covers less. Remember Nadiya does not qualify for Medicaid, Social Security or Rare and Expensive medical (REM).  The therapy we get for Nadiya is through school and through our insurance.

The Bone of Contention

Nadiya and her teacher in a bounce house
during Challenge Day

Many are probably wondering why we don't take Nadiya off the ketogenic diet. Yes it is true that the diet has undesirable long term effects that could prove harmful but for us the alternative is worse. David said it best many years ago when we were just starting out on this journey. He said, "It is our job to provide the best quality of life that Nadiya is capable of having. It will be different than that of Anastasiya or other typical children." Those words have guided me through many difficult decisions and have kept me focused on what matters. David and I have always believed in quality over quantity. For Nadiya to live in an unaware sedated and greatly impaired state until she is a100 years old is not a good quality of life.

Before the Ketogenic Diet, Nadiya's quality of life was very poor.  Her personality was locked in a body that was overmedicated and a brain that was buried in seizure activity.  Every time she would meet a milestone or make a gain she would lose it to a huge Grand Mal Seizure (Tonic Clonic Seizure).   By the time she would regain the skills she would have another debilitating Tonic Clonic seizure.  When Nadiya was riddled with seizures she could not look at us or bond with us.  She slept all of the time and rarely interacted with her world.  Her quality of life was very poor and her health was unstable.  We can’t imagine going back to that. 

Nadiya’s Infantile Spasms progressed into Lennox-Gastaut Syndrome (LGS).  Left untreated those seizure types associated with LGS can result in Status Epilepticus which is a life-threatening condition in which the brain is in a persistent state of seizure.  This could be a seizure lasting longer than a half hour or having clusters of seizures within a short period of time.  When Nadiya was 18 months she reached Status.  For her it was a Tonic Clonic seizure that lasted over an hour followed by a shorter seizure within minutes of the first one resolving.   This is serious and dangerous.  Uncontrolled Epilepsy can also result in Sudden Unexplained Death in Epilepsy (SUDEP) which is when an epileptic dies suddenly after a seizure from respiratory failure or cardiac arrest. 

These are serious risks for having uncontrolled seizures.  We feel it is most important to manage the seizures.  For Nadiya that means staying on the Ketogenic Diet and taking a low dose of Lamictal and an adequate dose of Depakote.  LGS is one of the most difficult to manage seizure disorders.  The fact that we found a way to manage it means everything.  It is worth managing the adverse and long term affects of these successful treatments which we will do.  I am not sure what that will mean for managing Nadiya’s bone density concerns but I will be prepared for whatever that will entail.

Nadiya walking in her kid walk

This is a lesson to all of us.  Talk to your dermatologists and primary care physicians about your vitamin D and Calcium intake.  Make sure you are getting enough weight baring activity.  Know your medications, their side effects and their risk to your bone health.  Get a Dexa Scan.  At the least it will create a baseline and at best it will alert you to a problem before it gets out of hand.  If you have a child with circumstances similar to Nadiya it is very important to be aware of their bone health.  Nadiya has come way too far to stop now.  I still hold a realistic hope that she will walk some day without a kid Walk and without adult assistance.  The first step to that is protecting her bone health.