Inchstones and Awareness

Marek had a a really good day on Wednesday. He only had 4 spasms. Of course we got our hopes up and were really excited that maybe, just maybe this might be the turning point. Unfortunately, Thursday night he had a big seizure of 73 spasms in 45 minutes. So as of now we are just focusing on getting his strength up and working on his development. Marek has had some rough days while weaning off the ACTH. A lot of parents that I have spoken to said that their children were more worked up and irritable while weaning because their little bodies are actually going through withdrawals of the drug. On top of that Marek has had quite an upset stomach and now has thrush in his mouth (one more drug to add to the mix). Wednesday night and Thursday night he hardly slept. We were back out taking middle of the night drives to calm him down. Last night though was a great night for him. Ryan and his mom watched him so I could catch up on sleep again and he slept for 2-3 hours at a time. Hopefully tonight will be another good night for him.

As far as therapy goes, our little man is working his butt off! We are excited that he can finally hold up his head while on his stomach. Granted he is only doing this on his "wedge" which keeps him at a 45 degree angle, but its a start! It's not a milestone but its an inchstone and we are happy to celebrate all the little moments. Marek has a new motto, which is "WILL WORK FOR FOOD!" He is doing so many little things that are making him stronger as long as we reward him with a bottle. He is able to get his shoulders off the ground when he on his back reaching side to side for his bottle. Our therapist showed me how to help him with rolling on an exercise ball. He is doing a great job understanding how to start to roll but he is just not strong enough to get there by himself. He has realized this past week that he has back muscles. All he wants to do is arch his back to see what is going on behind him. Our therapist was very proud of this but now we need to work those tummy muscles! He is doing a lot of sit ups this week so we can balance out those back muscles.

Thank you again for everyone who continues to support Marek on his journey towards a seizure free life. We also want to thank everyone who has come to the house to drop off food for us. We really appreciate it since we don't have much time or energy to cook since our focus is on Mighty Man.

I started this blog as a way to raise awareness of Infantile Spasms/West Syndrome and to help families that are going through difficult times with this disease. I have learned many valuable lessons in the short time going through this ordeal with Marek. One important thing I have realized is that support goes a long way. Support from family and friends, support from other families who are going through the same fight we are, and support from organizations. Today is National Rare Disease Day. There are over 6,000 rare diseases in the world and less than 500 treatments to help cure or control them. Many diseases don't have ANY treatments. Without awareness this will continue to be a problem. Not only is my son struggling with a rare disease but so is my dad (who has sarcoidosis). For both of them I will help to spread awareness with hope that one day drug companies start to take interest in the FEW but very SPECIAL people that struggle from these diseases. I ask that you take the time to watch the video and spread the word to just a few people and ask them to talk about it with a few more people. The ripple effect is a powerful tool. The video was put together by NORD (National Organization of Rare Diseases). They do so much to support families that struggle with rare diseases.

Ryan, Marek, and I have been blessed to receive the help of a student group at Collinsville High School called Kahokstrong. This student organization helps families of students and staff that are struggling. This year they are hosting their second annual Kahokstrong 5K. The theme this year is "Who is your hero?" Participant are asked to dress up as their favorite superhero. My family will be walking  to support our little but mighty superhero Marek as well as to support the club who has done so much for so many families. Please join us to help support a great and worthy student organization. We will be wearing our Team Marek shirts!

KAHOKstrong 5K

Monday Mom and I will be heading to Detroit to see Dr. Chugani. I will update everyone when we get back from our appointment.

A New Path

Sadly the doctors feel that the ACTH is not going to be Marek's miracle drug. We are weaning him off of the steroid and looking toward our next option. It is frustrating and very discouraging that the number one drug out there to fight IS didn't cure Marek of this disease. Marek will not be completely off of ACTH until March 17th. Since ACTH is so strong and Marek is still at risk for many of its side effects we don't have many options on what we can do next until he is 100% off the drug. Although we are off of the drug mid March he will not have a full immune system for many months. The doctors told us for the six weeks after ACTH he will still have a severely compromised immune system.

We met with our neurologist and epileptologist today at Children's to map out where we go next. It is scary because we are running out of options as far as drugs go and we have already tried the "top" treatments for IS. The plan our doctors propose is to continue Marek on the Onfi which he has been on for two weeks now. We will now be a little more aggressive with titrating up the dose as we slowly go down on ACTH. Before ACTH Marek was having over 200 spasms a day, ever since we have been on his high dose of ACTH he is having between 40-80 spasms a day. We are hoping that as we come off this drug his spasms don't become worse. If they do, then we will start another less potent steroid called prednisolone. If Marek's spasms stay in the 40-80 range we will start the drug Topamax (which Marek was on before and which caused his bicarbs to drop). If Topamax doesn't eliminate the spasms or if his bicarb levels drop again (we will have to go to Children's hospital every other day to get his levels checked) we will then be starting the Ketogenic diet towards the end of April. We will have to be admitted back into the hospital to start the diet. The doctors have scheduled Marek for an EEG on March 12th to evaluate if the ACTH has cleared up any of his hypsarrythmia. We are keeping our fingers crossed that it has.

Although we love the care we have received at Children's Hospital in St. Louis I have been trying to get a second opinion from a doctor in Detroit named Dr. Chugani. He has 35 years of experience with Infantile Spasms and is well known throughout the Epilepsy Community. We finally got a call today that they have an opening so we will be leaving for Detroit Children's Hospital Monday for our appointment on Tuesday. We feel that with any major medical concern a second opinion is necessary.

We have to thank everyone again for their donations and support. We are so thankful to have such amazing friends and family willing to help us. We will be using these donations to travel to Detroit and to be under the care of Dr. Chugani who is not in our insurance network. I have spoken to many parents that I have met through Infantile Spasms support groups who have gone to Dr. Chugani and have great things to say about him.

A new path to travel down brings new hope and new fear. We hope that this will be the KEY to stopping our son's IS and yet at the same time we are fearful that he will fail another treatment. In regards to treating IS there are four main treatments. #1 ACTH/Prednisolone #2 Vigabatrin #3 Topamax and #4 The Ketogenic diet. My family and myself are big believers in signs. I was so nervous with how today's appointment was going to go because of our timeline. Dr. Chugani's office told us that he would like to run a PET scan four weeks after Marek is off of ACTH which would be mid April. In order to have a PET scan done he cannot be on steroids or the Ketogenic diet. I was scared that our doctors in St. Louis would have a plan that would not allow us to have the PET scan ran in Detroit. So after hearing that Marek wouldn't be on steroid or the Ketogenic diet at that time (granted everything goes according to plan) I was so relieved and felt that we are finally heading down the right road. Our prayers these last four weeks have basically been on repeat asking God to let the ACTH work. Tonight I will have a new prayer asking him to allow our new path to grant Marek seizure freedom.

My aunt sent us a message today that said, "It is usually the last key on the keyring that opens the door." We are coming to our last key...fingers crossed the door opens.

Below is information about the Ketogenic Diet from the Epilepsy Foundation

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• What is the ketogenic diet?

The ketogenic diet is a special high-fat, low-carbohydrate diet that helps to control seizures in some people with epilepsy. It is prescribed by a physician and carefully monitored by a dietitian. It is stricter than the modified Atkins diet, requiring careful measurements of calories, fluids, and proteins.

• The name ketogenic means that it produces ketones in the body (keto = ketone, genic = producing). Ketones are formed when the body uses fat for its source of energy.
• Usually the body uses carbohydrates (such as sugar, bread, pasta) for its fuel, but because the ketogenic diet is very low in carbohydrates, fats become the primary fuel instead.
• Ketones are not dangerous. They can be detected in the urine, blood, and breath. Ketones are one of the more likely mechanisms of action of the diet; with higher ketone levels often leading to improved seizure control. However, there are many other theories for why the diet will work.

Who will it help?

• Doctors usually recommend the ketogenic diet for children whose seizures have not responded to several different seizure medicines. It is particularly recommended for children with the Lennox-Gastaut syndrome.
• The diet is usually not recommended for adults, mostly because the restricted food choices make it hard to follow. Yet, studies done on the use of the diet in adults show that it seems to work just as well.
• The ketogenic diet has been shown in small studies (case reports and case series) to be particularly helpful for some epilepsy conditions. These include infantile spasms, Rett syndrome, tuberous sclerosis complex, Dravet syndrome, Doose syndrome, and GLUT-1 deficiency. Using a formula-only ketogenic diet for infants and gastrostomy-tube fed children may lead to better compliance and possibly even improved efficacy.
• The diet works well for children with focal seizures, but may be less likely to lead to an immediate seizure-free result.
• In general, the diet can always be considered as long as there are no clear metabolic or mitochondrial reasons not to use it.

What is it like?

• The typical ketogenic diet, called the "long-chain triglyceride diet," provides 3 to 4 grams of fat for every 1 gram of carbohydrate and protein.
• The dietician recommends a daily diet that contains 75 to 100 calories for every kilogram (2.2 pounds) of body weight and 1-2 grams of protein for every kilogram of body weight. If this sounds complicated, it is! That's why parents need a dietician's help.
• A ketogenic diet “ratio” is the ratio of fat to carbohydrate and protein grams combined. A 4:1 ratio is more strict than a 3:1 ratio, and is typically used for most children. A 3:1 ratio is typically used for infants, adolescents, and children who require higher amounts of protein or carbohydrate for some other reason.
• The kinds of foods that provide fat for the ketogenic diet are butter, heavy whipping cream, mayonnaise, and oils (e.g. canola or olive).
• Because the amount of carbohydrate and protein in the diet have to be restricted, it is very important to prepare meals carefully.
• No other sources of carbohydrates can be eaten. (Even toothpaste might have some sugar in it!).
• The ketogenic diet is supervised by a dietician who monitors the child's nutrition and can teach parents and the child what can and cannot be eaten.

What happens first?

• Typically the diet is started in the hospital. The child usually begins by fasting (except for water) under close medical supervision for 24 hours. For instance, the child might go into the hospital on Monday, start fasting at 6 p.m. and continue to have only water until 6 a.m. on Tuesday. The diet is then started, either by slowly increasing the calories or the ratio. This is the typical Hopkins protocol.

7 Months Old

Our little peanut turned 7 months old on Friday. These last two months have been hell for our little guy but our super hero continues to amaze us each and every day . He has been through more in the last 7 months than many have been through in their entire lifetime. His strength and bravery is unbelievable. I see him working so hard on all of his skills and I know that he has it in him to conquer every obstacle that stands in his way. His laugh and smile are what gets me through each day. Even through this horrible drug treatment he has been strong enough to learn new things (like hold his bottle). We are hoping that this will be the month that his strength and determination will pay off and he will become seizure free.  We want you to know Marek that Mommy and Daddy are behind you 100% and we have all the faith in the world in you. You will beat IS. Happy 7 Month Birthday!

Day 21 of ACTH

I think I can finally write a post to update everyone on Marek's fight. It has been too hard for me to sit down and put into words that things haven't been the best. Just thinking about things brings me to tears. Anger, frustration, sadness, helplessness, fear exhaustion are just a few of the emotions that overwhelm our family every day. Although it is hard for me to share Marek's journey at times I know that I have to. I have to because for one, so many friends and family are sending their love, prayers and support to Marek and we know they are traveling right along with us on this journey and I don't want to keep them in the dark. And secondly I want this blog to help spread awareness of Infantile Spasms and hopefully help other families that are suffering from this ugly diagnosis.

Let me back up to this weekend. On Friday Marek started a new drug called Onfi. He was on 1 ml once a day and as of yesterday he has gone up to 2 ml a day. His side effect have been pretty mild on this drug. He is drooling a ton and he is "out of it" a lot of the time but other than that he has tolerated it pretty well.

My mother-in-law came over Friday night to help Ryan watch Marek overnight. Marek was happy to see his Gigi. By Carol coming over, my mom and I were able to finally catch up on some sleep. Marek kept his daddy and Gigi busy from 2am until 8am. They spent some quality time in the bathroom while Marek played in the tub and in the car driving around to keep Marek calm. My mom and I are so thankful to had a night to "recharge."

Saturday we received a letter from our insurance company that is still making my blood boil. They told us that they denied our claim for all therapies for Marek because his "prognosis and/or history does not show reasonable chance of developmental progress."

Dear Mr. Big Shot Medical Director (who by the way is NOT a neurologist, pediatrician or epileptologist) you have no idea what my child is capable of and we don't appreciate you telling us that their isn't hope for his future. Marek is one tough little boy and will do amazing things in his life. He will have to work twice as hard as many of his peers but with therapy (even without the assistance of insurance) he will get over all the hurdles standing in his way. 

I wanted to immediately drive to his office after reading that letter but not only could I not find where he was located I couldn't even get a hold of him. What is even more frustrating is neither could our Neurologist (who laughed when reading the denial), our pediatrician nor our therapist.

Monday was exciting because we got to spend an extra day with Ryan due to the weather. Marek was very happy about this and showed it through many smiles and small belly laughs that day. We also were given the go ahead by our Neurologist to keep Marek at a high dose of ACTH until tomorrow. We wanted to make sure that we did everything we could while on the ACTH to help him. Unfortunately, it hasn't helped. Marek is still having spasms. Thankfully he is not having as many as he was before ACTH but the medicine hasn't done what it should. It is scary to think that the most powerful steroid out there wasn't able to help our son, this was the #1 drug at fighting IS and our son isn't responding to it. I don't understand, I never will. I ask myself every day, "why Marek?"

Wednesday we had Marek's first physical therapy session with Patti. We absolutely love her. We feel so blessed that she was the one chosen to work with Marek. She was scheduled for an hour and stayed for almost three hours. She brought us a bunch of equipment that we get to keep here at our home so we can work with Marek during his "good times." Marek did a great job at therapy. I honestly thought it was going to be a disaster since therapy was scheduled at 8:30 am and he had been up almost the entire night, but he was a trooper. She showed us many sensory exercises to do with him to calm him down. These sensory exercises allows him to get the most out of his strength exercises since he isn't crying or fighting us. Patti has also been amazing with going above and beyond to fight our insurance company. We can't thank her enough.

Today, we had an appointment with the eye doctor. During our first hospital stay they did an eye exam on Marek and said that one of his nerves in his eye seemed slightly smaller than it should have been. They weren't sure then if it was going to cause issues for him or if it was just a stage of development and it would grow with time. Therefore, they scheduled his appointment for today to check the development of his nerve. During Marek's first steps evaluation the therapists kept mentioning that they thought he had vision problems. I have started to think that his vision problems were being caused by this small nerve. After the eye exam today we found out that Marek's eyes are perfect, the doctor didn't notice anything wrong with his nerve this time. However, he did diagnose Marek with Cortical Vision Impairment. What this means is that his eyes can see perfectly fine but when images are transferred to the brain, his brain is having a hard time deciphering what they are because of all the seizures. So Marek isn't "seeing" or "tracking" things as he should be at his age. The doctor said that if we can get the spasms to stop there is a good chance that his vision can go back to normal. PLEASE LET THESE SEIZURES STOP!

We will have to make a lot of decision this week on where we should go from here as far as treatment goes. Everyone's continuous prayers are appreciated. I wish I knew why God is letting these spasms continue to hurt our child, sadly I know that question will never be answered. I can just hope and pray that he allows something to help our son in the near future.

Day 18 of ACTH

Still having spasms :(

Day 14 of ACTH

I will start with good news this time. I reordered Marek's ACTH yesterday and they told me it would be here today. It took everything I had not to laugh at that while on the phone with the representative. But to our surprise the doorbell rang at 9:30am and his remaining three vials of ACTH arrived (it still amazes me that these little vials cost $28,000 each). I can now breathe a little easier knowing he will not be without the drug.

Now to the not so good news. Marek had a rough night last night. He woke up at 11:30pm and didn't go back to sleep until 5:30 this morning. He was so uncomfortable all night, he continuously grunted and cried. We tried a bath at 1:30 am which seemed to work. While in the tub he was content. He took an hour bath and then we fed him, as soon as he finished his bottle he went right back to crying and grunting (as if he was in pain). We tried giving him a bath again at 4:30 am but this time it didn't work. We felt so helpless trying to calm him down. We tried Tylenol and Orajel just in case he was uncomfortable due to teething, but that didn't put a dent into his tantrums. Once he finally fell asleep this morning he only slept for two hours.

We are still on this awful seizure roller coaster. We have a good day where we think "okay maybe this is it" then the next day he is having 20 minute or longer seizures. It is frustrating because I have read so many stories where ACTH has stopped the seizures after the first injection and I can't help but feel jealous of those families and wonder why doesn't Marek deserve that? It makes me so angry! We have one more day at high dose, I have to stay hopeful that maybe tomorrow will be Marek's day for the medicine to finally work. When this whole nightmare started I thought to myself, "I have never been so scared in my life" and that was when we had every medicine that has been proven to be successful with treating IS in front of us. I retract that statement, "NOW, I have never been more scared in my life." We have tried and failed more drugs at this point than there are left out there to still be used to treat IS. We really need ACTH to be our miracle.

Things are getting increasingly harder to bear as we move forward with ACTH. Marek's irritability is at an all time high (I am writing this post from our bathroom where I spend most of my time since it is the best way to soothe Marek). I only get to see a smile from my son every few days now. I am in disbelief at how quickly things change. Just Monday he was socially interacting with his therapists and now he just stares into space in the moments when he is quiet and then will start another grunting and crying fit. His poor little brain isn't getting any rest since he is never asleep. Mom and I drove all the way to Lake St. Louis today and back just to help him nap which was a three hour drive. As far as the injections themselves go either Marek is getting smarter and now knows that when we put him on the counter and give him a bottle that he is going to get pricked or his poor not so little legs are starting to get sore from all the injections because we cannot keep him content during his shots anymore. He screams through each one now making it twice as hard to give them to him. Like I have said before I just want all of these horrible things that are associated with this drug to be worth it for Marek's sake. I will gladly stay up all hours of the night, drive all over this country and spend my whole life sitting next to a bathtub watching my son soak if I could be promised that he would be cured of IS.

Yesterday I emailed Marek's doctors a list of questions and concerns because I have started to question whether his medical team is being as aggressive as they should be in treating Marek's IS. I told him that our research on IS has lead us to families who have had success with "drug cocktails". I told our doctor that we want to add another medicine to Marek's treatment plan. I also asked how many children respond to ACTH after the weaning process starts? His neurologist finally called me today to discuss our concerns. After consulting with the other Neurologists at Children's he is going to start Marek on Clobazam tomorrow (IF insurance cooperates). He told us that Clobazam is not a magic bullet when fighting IS but maybe it will help the ACTH clear up Marek's Hypsarrythmia. In regards to how many children respond after the wean, he told me "its hard to say." I still have no idea what the hell that means!!! Staying on the optimistic side (which is hard to do every day) I will translate it as there is still hope. He ended the conversation with me by saying that he would not consider ACTH a failure yet. I am to check in with him tomorrow morning to see if our new drug was approved by insurance and to schedule a time to pick it up. FINGERS CROSSED! Marek's epileptologist got back to me this evening and told me he rarely sees patients respond to ACTH later on but has seen a few cases in which the patients respond at the end of week two or early week three. (End of week two is tomorrow for us). However, he did discuss an option on ACTH that we will be discussing with our neurologist tomorrow morning as well.

Ryan told me that there was an amazing turnout at Applebee's yesterday. Thank you to my CHS family for organizing that and to everyone that went out to eat and to show their support. I have to keep telling myself over and over that it is impossible for ACTH not to work when there are so many people out there praying for Marek. Please keep the prayers coming, a seizure free tomorrow would be the best blessing in the world. We love everyone that has been behind us during this fight!

Day 12 of ACTH

There really isn't much to write about today.

Marek is still having spasms. His last high dose of the medication will be given this Friday. We are hoping to see a miracle in the next few days.

Marek was evaluated by first steps on Monday. He had a Physical Therapist, Occupational Therapist and Developmental Therapist come to the house. I was very impressed by all of them. Marek qualified for therapy in all areas. He was higher is some areas than in others. His highest score was in social interaction and they put him at a 4 month level. His lowest scores were in his gross motor skills and fine motor skills where they put him at a 1 month level. He will get his first therapy session next Wednesday.

We are seeing the side effects of ACTH in full force now. Marek doesn't sleep for more than an hour and a half at a time (day and night). When he awakes from these naps he gets into screaming and grunting fits where he won't calm down unless we feed him, give him a bath or put him in the car. Yesterday Mom and I drove around for three hours to keep him content. He also took three baths during the day and two at night. My mom gave him a bath around 6 am and he fell asleep in the tub so she just let him sleep for an hour while watching him since he was so peaceful. We just want him to be comfortable. He is eating 6 ounces every 2 hours. He is constantly hungry which we knew would happen on ACTH but he will keep eating until he throws up. Therefore, we have to limit how much he has which just makes him more upset.

We are hoping that all of these side effects and misery we are putting him through start to pay off...I don't know what I would do if these injections were all for nothing.

Fingers crossed and staying hopeful!

Still holding our breath, waiting, hoping and praying.

Still holding our breath, waiting, hoping and praying.

On Thursday, Ryan came home to stay with us until Sunday. It is so nice to have him here, especially at night. I needed a new shoulder to cry on, my mom's needed a rest. Marek is also enjoying having his daddy back. Marek loves to cuddle with me and I seem to be the only person that can calm him down when he gets into his crying fits but his daddy is the one that can get him to belly laugh. It was so comforting to hear that again.

Marek is starting to "fill out" due to the steroids. His appetite has increased and his little cheeks are starting to bulk up. Last time the nurse weighed him he was 21 pounds. He is in 18 month clothes right now because of his "little" tummy being too big to zip up some of his 12 month sleepers. Even extra chubby he is still the cutest peanut I have ever seen.

Thursday night our priest came over in the evening to pray with us and to perform the anointing of the sick. It was very emotional for all of us. I wish I could have screamed our prayers because maybe God would have heard us a little better. Father said he would pray for Marek in each of the masses this weekend. We are hoping maybe with a few more people on board praying we can tip the scale in the right direction, who knows?

Marek sleeps from 9pm-12am and then is up from 12am-6am every night. I usually take the over night hours and then my mom takes over at 6am so that I can catch up on sleep in the morning. On Friday, after a long night up with him, our nurse came over around 8:45am. I thought that it would be just a normal visit and I could go back to bed. WRONG! She took his temperature and he was running a fever. My greatest fear, besides not having the spasms stop with this medicine, is that he becomes sick and can't fight his infection since he is immune suppressed. I called Children's Hospital and talked to our neurologist who made me feel a little better. He didn't want to admit us to the hospital since it is a germ factory so he told us to monitor the fever here at home. He told us not to administer Tylenol so that we could get an accurate reading and nothing would be masking his temperature. We are continuing to take his temperature every four hours. Luckily it hasn't increased at all so we have been able to stay in the safety of our own home.

Last night Ryan and I took the night shift together and I was so happy he was here because Marek had his longest seizure to date. After an hour of watching our poor baby having spasms Ryan called Children's Hospital to see what we should do. They told us that there was nothing they could do to help him. Infantile Spasms seizures do not respond to emergency anti-convulsant medicine. The on-call neurologist told us to just watch him and wait it out with him. I am SICK OF WAITING AND WATCHING! We want to help our son! We want the doctors to have an answer for us and a foolproof plan that will give us the results we so desperately earn for. IS THAT TOO MUCH TO ASK? Marek was so exhausted from his seizure that his eyes were closed and he would try to go to sleep but then he would have a spasms and his eyes would pop open and his body would jerk. And of course we just sat there and watched. It makes me sick. After 1 hour and 17 minutes his seizure finally stopped. He had a bottle and then went to sleep.

It is getting harder and harder each day. Today is day 8 of being on ACTH and still he is having seizures...HOUR LONG SEIZURES. It is discouraging and I get angrier and angrier each time it happens. I think days like today are probably the hardest. Everyone I am sure is excited that it will be a beautiful, warm sunny day out, but I don't like the sun right now. It doesn't match the situation we are in. It should be overcast, stormy and freezing cold. I hate that life just goes on all around us while we are stuck in this horrible hell hole. Like many, I used to scroll through my Facebook newsfeed to catch up on what everyone else was doing but I can't bring myself do to that. I hate seeing everyone's pictures of their perfectly normal healthy kids, hearing about wonderful news going on in their lives or even more upsetting people ranting on Facebook about trivial matters. Instead, I go on to Facebook to visit the Infantile Spasms support groups because people posting on there are currently in or once were in the same horrible hell hole as us. I know life goes on outside of our home, but right now I don't want any part of it.

Although I am in a really crappy mode today and don't want much to do with anyone I am very thankful for everyone's support, love and prayers. We have had wonderful little surprises show up at our front door every day (Thank you Snodgrass Family), my sister has come to visit through the window and run countless errands for us (Thanks Sis), we have had food made for us and dropped off by so many people (Thank you ALL). Our families have been amazing through all of this, we have the best parents in the world!

Finally Home

We came home from the hospital yesterday. My dad stayed at our house and waited by the front door until the medication came. Once we had the medication in hand they released us from the hospital.

The whole process to get this medication approved by insurance was hell and then the pharmacy dragging their feet on "processing" the medication was enough to make my family go insane. Once again we were left feeling helpless because there wan't anything we could do to get the medication here any faster. We called the pharmacy three dozen times a day and all they would tell us is that it was "processing". We looked up the pharmacy online and the reviews were awful. Poor customer service, long "processing" times and even patients with cancer not getting their medication for days. Accredo pharmacy is a joke. I called every contact person that I knew, both on the insurance end and the ACTH manufacturing end, and both told me that this is the only pharmacy WE could use. I have zero faith in this company and what scares me even more is that we will have to refill this medicine three times before the treatment is complete. I pray that they get us our refills on time.

We are so glad to be home! The hospital was a scary place to be knowing that Marek's immune system was slowly dropping. I watched the news every day and hearing about the measles outbreak and how the flu shot isn't effective this year made me want to cry. Marek was not able to get his 6 month shots because of his compromised immune system so we will have to be very careful, even after this is all over, until we can resume his vaccinations. The hospital was also difficult because he wasn't sleeping all that much since he was constantly being woken up in order for the hospital staff to examine him, check vitals etc.

Marek is receiving his highest dose of ACTH now. He gets injections twice a day until the middle of March. We are now on "lock down" in order to keep Marek safe from germs. As far as side-effects go he is doing great on the medicine. His glucose levels were fine at the hospital along with his blood pressure. Our home healthcare nurse comes today to take his blood pressure here at the house. She will be visiting us three times a week to monitor him. As far as irritability goes, at first he was sleeping more than he was awake and seemed pretty happy but starting Sunday night we saw a change in him. He is becoming more and more fussy. The last few nights he just cries and fusses from midnight until 4am or 5am. Nothing seems to settle him down. We know he is exhausted because he is fussing even with his eyes shut.

We haven't seen a change in his spasms. Yesterday was a rough day because he had three seizures that were all close to 30 minutes in length. I couldn't bring myself to update everyone when all I wanted to do was crawl into a hole and not come out. I keep waiting for a sign that this medicine is working. I plead with God each night, he can take anything from me, do anything to me if he could just let my baby come out of this okay. It's hard at times, especially at night because I am begging him to help Marek but I feel that my prayers are falling on deaf ears. My heart feels like it weighs 1000 tons and the pit in my stomach keeps growing larger every day. I have never been more scared in my life. I try to console Marek when he is having a seizure telling him everything will be okay but then I feel guilty saying that because I can't promise him that. I can't change anything, I can't help him, I can't even be strong for him at times. All I can do is watch him while he has a seizure knowing that so much is going on in his sweet little head that is hurting him. Every day I feel more and more defeated. Every day I hope that he won't have a seizure and when he does the disappointment that washes over me is overwhelming. It's as if the hope I have that the medicine will work lifts me up to the tallest mountain  but then when he has a seizure it's as if someone pushes me off that mountain and I fall all the way into the deepest valley. Picking myself up after each of these falls is getting harder and harder.

So as of now we continue to wait and watch. Marek is still the bright eyed little boy that he always has been. The medicine hasn't taken away his smile or his heartwarming belly laugh. (ACTH is known for making kids go into a sort of shell where they don't interact at all) We are watching his developmental skills closely. When he is in a good mood (after a nap and full belly) he will still reach for toys, babble and play. However, those good moods don't come as often as the used to.

Thank you again for everyone's support, love and prayers. We love our little guy so much and all we want if for him to get better.