O my goodness do I have so much to tell you. I really have a lot to talk about because there is so much going on. In fact, my head is still spinning after all that has happened today. I can’t believe all that is happening. Perhaps once I put all that is happening on paper (well actually into the computer), maybe I will get a grip on reality and maybe I will better be able to handle what is going on and know what to do because at this point, I am ready to go bananas. I have to come back here to have really hard, radical and life-threatening surgery in 2 weeks and I am really freaking out. I never thought I would be having the surgeries and procedures that they told me about today. You know things are bad because dad let me go and buy things after my appointment without even questioning me as to “do you really need it?” like he usually does. I never saw my dad like this, so you know things are bad.
Well… I did make it to John Hopkins Hospital in Maryland with dad. When we arrived at the hotel, we found out that not only were we given the discount that we were already promised, but they also added an additional discount to our stay, which made our stay cheaper than we originally thought it was going to be. So, the day started out being great because of course saving any money is a good thing because we really don’t have money to spend fruitlessly, as the bills that we have are stacked up. We really have to watch every single penny because my treatment is so very expensive and it does cost a lot to be able to go and get it too. So… any penny saved is definitely worthwhile and a HUGE plus because it is extremely helpful.
The room was simply gorgeous. Actually the whole entire hotel was magnificent. It was located right on the harbor and it connected to so many things to do such as shopping, Ripley’s Believe it or Not, Restaurants, etc. Even the aquarium was not too far or even the Hard Rock Café. Our room was even magnificent. We were fortunate enough for the manager of the hotel to give us a room overlooking the harbor at no added cost. I was really happy because I knew that I probably wouldn’t get to enjoy going to the harbor as much as I would have liked. By having this room, I would be able to overlook the harbor and watch the boats and everything. Nothing could compare to the view that we had. It was simply amazing.
The room was really nice and it was set up very nicely. However, it had this one device by the bed that dad couldn’t figure out what it was. When I told him it was a sound device to mute out the noise and everything, he thought that I was joking. However, when the valet people bought up our suitcases, I questioned them and they confirmed that it was indeed a sound device because it could get very noisy even though we were on the 12th floor because the nightlife here is amazing. I told my dad that it was to mask his ‘snoring’ because we all know how badly he snores and how much I can’t take it. I spend the whole night literally beating him up because he snores so loudly and I can’t deal with it.
Dad also said that it was “my day” to do whatever I wanted. Knowing how it is so beautiful here in Maryland and how our hotel is sitting right on the inner harbor, I really wanted to take advantage of the area. However, my disease really doesn’t permit me to do as much as I would like. My dad and I went out and walked around the harbor, but I really would have loved to go paddle boating or even go to “Ripley’s Believe It or Not!” I really would have even loved to stay out longer than we did, but like always… my disease ruins everything and prevents me from having a good time. My dad did say that he would take me “paddle boating” because he knew how much I really wanted to do it, but lets be frank about it… how would he manage to ‘paddle’ both us around by himself? So since it wasn’t ‘free’ and wasn’t ‘cheap.’ I didn’t want to see him waste his money on this to find out that he couldn’t do it all himself because honestly I felt that it was going to be an impossible feat to ‘paddle’ all by himself because why else would there be peddles on the boat meant for 2 people? I didn’t want to chance it.
In addition, the sun, heat, and mugginess really did flare up my illness and caused it to really become worse than ever and as a result, we were forced to go back to the hotel. I am extremely in pain and highly hypersensitive. Therefore, the sun was burning me because it was way too strong and it was too hot outside too. Plus, with all the mugginess of the weather too, it just was not a good combination. It just really flared everything up and it felt like my pants were on fire. When I get hot, I cannot sweat because of the autonomic dysfunction and as a result, I end up getting prickly and blistering. It was nothing but a disaster, but dad was my usual hero and came to my rescue. He took me back to the hotel and tried to make me as comfortable as possible.
I was really disappointed that I didn’t get to do all the great things that I wanted to do. I really wanted to go to “Ripleys” but the heat got to me and I would have loved to go to the HARD ROCK CAFÉ to get a sweatshirt, but it was too far of a walk. Maybe we will get to do these things when we come back the next time.
I do have to say that I was impressed that on my outing that I saw these solar power trash compactors. I couldn’t believe that there was such a thing to be honest with you. I wonder why there wasn’t more of them around like in New York.
The day finished up by going for dinner and having problems in that area too. I didn’t even want to go for dinner, but like always, my dad made me because he said that I couldn’t afford to “lose weight.” Due to my illness, I can’t eat and swallow many foods and the ones that I can need to be prepared a certain way. I ended up having to send back my food so many times because it wasn’t made how I needed to it be that we ended getting our dinners free. If my dad would have known our dinners would have been “free,” I am sure that he would have ordered something else.
My dad usually orders meals when we go away based on the cost. When you are strapped for cash and watching every penny, the cost plays a huge factor in whether you get something or not even though you might want something else. My dad much rather have wanted something else I am sure at this restaurant that we ate at tonight, but he ended up getting a turkey club. He should have had some seafood or especially crab or something considering that we were in Maryland and it probably would never have been better than if he got it here, but he ended up going with the Turkey club because of the cost. However, if he knew that the dinner was going to be free, he probably would have went with a better dinner such as the seafood or something.
When we were sitting at dinner, my dad said to me that the chef was looking at me. I saw the chef come out, but I didn’t think my dad was really serious. Well… no sooner did he say that but the actual chef came out of the kitchen with my food. We had sent my food back so many times that the actual chef came out to personal hand deliver the food and to make sure that it was made to my liking and to also apologize for everything that had occurred. I couldn’t believe it. But to further add to our surprise besides the chef coming out, they ended up not charging us at all and the bill wasn’t cheap either.
But that was yesterday and today didn’t go too great and was filled with so much bad news (well good news depending on how you want to look at it). I should have known something was going to happen from the moment that I woke up because first of all we overslept when we couldn’t afford to because today was filled with all my doctor appointments at the hospital and the weather was also nasty outside. We didn’t set a wake up call because we thought we would definitely have been up because my dad never sleeps late. We didn’t have to be at the hospital until 9AM, so ideally we didn’t have to be up until 7 AM because we would have to get dress and grab breakfast. There would be no way that we would need a wake up call because dad never sleeps that late.
But I guess dad was just so exhausted from all the hard work that he has been doing at his job and such and with all the stress that he has been under because he actually overslept. If it weren’t for my mom, we would have definitely missed our appointments. I never sleep either, but right before 7AM, I ended up taking my meds, which always knock me out for a brief period of time. When I take my meds, it knocks me out for about 30 minutes, which is the only ‘rest’ I get. Of course when I wake up, it is back to pain and I am up for the duration. But thankfully, my mom called us at a little over 7AM to say “Good Morning,” which woke us up so we weren’t late to the hospital.
What a day we had at the hospital! I am really glad in a way that we came to John Hopkins because I am really happy with my new team of doctors here even though I am extremely exhausted and fearful about all that is about to happen. I never thought that I would have to undergo all that is going to happen, but I hope that I am in good hands because I am at John Hopkins. After all, they are ranked like the #1 hospital in the country. Never before did I feel so comfortable at a hospital. I really loved the team of doctors that I met with. They were incredibly not only incredibly knowledgeable and willing to help me, but they were also incredibly nice and had excellent bedside manners. They really made us feel extremely comfortable and took their time with us. They didn’t just “rush” us in and out. They answered every question that we had no matter how stupid and repetitive it was. They really made us feel “welcomed.”
Unfortunately though, the doctors did say that I am way too sick to be helped right now. They said that in the past, they have been known to hold people in their office for hours if it meant that they could help them and do something. However, in my case, they said that it would even be impossible to do anything at all if they held me all day because I am so medically sick and medically complicated. As a result, they said that I needed the help that went far beyond their hands. They said that I needed the work of the entire hospital, as I needed the help of specialists from various disciplines across the entire hospital spectrum. Therefore, they said the only thing that we could do was be hospitalized in order to be actually helped. They said that what needed to be done required not only to be hospitalized but because it was so life threatening as well, I would have to be placed into the ICU. It couldn’t even be the “step down” ICU, but it has to be the very ICU itself, which is extremely difficult to get into because there are only so many beds.
As a result of it being extremely difficult to get into the ICU and because they are going to have to schedule all the surgeries and procedures that are going to have to be done, it is all going to take some time. In addition, since the upcoming procedures and surgeries are going to be extremely life threatening and dangerous, I really want to go home and see my mom before they are done just in case something does in fact happen. Therefore, they said that while they get everything organized (which will probably take about a week or two), I should go home and enjoy myself and spend as much time as I can with the people that I love. After all, what I am going to have when I come back is extremely dangerous and scary.
I am really surprised that they are letting me home because with all that they told me that they are going to do, I would be afraid that my patient would not want to come back. I mean… to be honest… I am really starting to get cold feet just knowing all that is going to have to be done. I am not going to lie, but I am extremely nervous. But I know that it is something that has to be done or I am going to die. It just stinks that this is so very dangerous and can kill me just as well. But I guess I have no other choice and something I am going to have to chance. I just hate that I am going to have to miss my mom’s birthday too because when we come back it will be the time that my mom will be celebrating her 60th birthday. With all that she has done for me and has given up, I really wanted to make her birthday special and grand. I really hate that I won’t be around to do that for her.
Unless something happens sooner, we are supposed to be heading back to John Hopkins June 28th. We were already instructed that if I was to feel any worse whatsoever, we were to come to the ER immediately. Hopefully I will be able to last though just like I have always managed to do so. I really don’t want that day to come because I am so very scared, but we all know that the more you don’t want something to come, the faster it will. So… I am sure that day will be here in no time.
When we come back, there will be so many surgeries and procedures that I will need to go for. They said that I am so very sick and my GI tract is so ‘dead’ and sick that it is overly stretched and the nerves are just ‘shot.’ I questioned them if it was normal to be experiencing the symptoms that I have been experiencing for my illness and they totally said it was. I never feel that I am ‘full.’ You know when you eat how you gradually feel fuller and fuller until you reach capacity and feel completely full? Well I never get that feeling of feeling fuller and fuller. I always feel hungry until that split second when I feel overly full. It really stinks because I am so hungry and then I get slammed because I feel just overly stuffed. When that happens, I get overly distended, I vomit, and my autonomic dysfunction starts up. There is never a dull moment with me.
Since my GI tract is so bad, I need radical treatment to be done. Of course the best thing that could happen would to get a new GI system, which would require getting the multivisceral transplant (new stomach, small and large intestine, pancreas and spleen). But this is extremely risky and I most likely won’t survive it. Therefore, we need to do other things in the meantime to try to buy me time and build me up to prepare for that transplant because most likely that will have to be the end result. However, if nothing is done in the meantime, I won’t last much longer because I am rapidly deteriorating and my organs are shutting down. I basically weigh nothing and have a BMI of 10.8. I really can’t afford to lose any more weight whatsoever.
The doctors also think that I have an obstruction or twist in the colon. However, due to the colon being so distended and stretched, it has kinda worked in my favor because it has allowed me to be able to live with this obstruction or twist longer than a typical person. So even though they would like to deal with this right now, they are hoping that I will be able to make it until my return visit before they do because any bowel or GI surgery is extremely risky and complicated. When you have any GI surgery, it is the hardest surgery to do because the chance of complication is extremely high. There is so much bacteria in the GI tract and therefore, it is the most risky surgery you can basically have. You really have to be strong to have any sought of GI surgery. So that is why they are hoping to wait until next time I come because this way I can have everything set up and such.
Therefore, when I come back, I am not only going to have this obstruction or twist looked into and taken care of, but they are going to be doing other surgeries and procedures too. One type of procedure that I am extremely scared about is that they are going to give me these types of medications that are extremely strong and potent and have the ability to really ‘reverse’ things in my GI tract. The thing is that on top of ‘reversing’ my GI tract, it will be stopping my heart as well. The doctors told me how these medications will stop my heart in a second and I will have to have my heart restarted by the paddles. It is through this restart of my heart and GI tract that my GI system is supposed to be ‘reversed.’ However, when they talk about ‘reverse’ all they mean is that it will go back a bit to being perhaps more tolerable. It won’t cure things. It will only mean that I won’t be so distended and it will make me feel better. It definitely should make a huge difference even though it won’t technically cure me.
I am extremely nervous because just knowing that they are going to stop my heart and need the paddles to restart it, it is freaking me out. I am so scared that my heart won’t restart or that my body won’t be able to take it. I am also scared that when they use the paddles to restart my heart that it will be so painful. After all, when they use the paddles, there is so much electrical current going through your body. That is why they yell “clear” and the body “jumps.” I remember when my grandma was dying and they were asking me about the “Do Not Resuscitate Order,” they said that she would never survive a resuscitation really because the paddles alone would hurt her and probably do damage. I am so scared that they are going to do the same thing to me. After all, I am so fragile and such.
If left untreated, my condition is extremely dangerous, as it may cause ischemic necrosis and colonic perforation, with a mortality rate as high as 50 percent. Therefore, they are going to do some procedures to try to help that as well. In addition, they are very concerned about putting weight on me to buy me time because I am so underweight and emaciated. I am extremely malnourished and they are worried that without any nourishment, I am not going to make it much longer. Plus, I am going to need nourishment in order to recover from all these procedures. I have no reservoir to help me out to fight any infection or to basically recover from anything. So, they want to put tubes in me as well.
Besides also trying another nasal-jejunal tube, which is a tube that they pass through the nose, through the stomach, and into the intestines so that they can try to find one small viable portion in order to somewhat give me nourishment, they want to try different forms of TPN. I have failed all these in the past, but they want to try different forms and mixtures that my body might better accept. But I am a bit leery because of all the other times I failed it. I am scared because I know how much this has stirred up the autonomic dysfunction and exacerbated my illness in the past. I really don’t want this to happen again. But I don’t know if we are going to have any other choice at this time. They are even talking about one cocktail even consisting of just breast milk. I feel like I am not even a normal person again between all the interventions and tubes.
On top of all this, I will be getting ketamine around the clock. Of course all this stuff definitely would not be possible without the ketamine because my disease spreads through trauma and you can bet your dollar how much “trauma” all this stuff is going to cause to me. Therefore, I am definitely going to need ketamine not only to hopefully stop the progression of the disease with all these surgeries and procedures that I am going to have, but it is needed because I am going to be in so much pain. Ketamine is really my “miracle drug” because it is a NMDA receptor and has the potential of reversing my disease and stopping the progression. Of course for it to really reverse my entire disease, I would need such a high dose of ketamine that is not FDA approved and not able to get in the United States. So in the meantime, I have been getting littler doses in hopes that at least it would slow down the progression and not spread as fast. I am also hoping that even though it won’t “reverse” the disease entirely, I am hoping that these littler amounts will reverse it a little. Any bit is little than spreading. You know? I have seen firsthand the miracle of ketamine because the last time I had a procedure I couldn’t even stand afterwards because my disease really did spread big time. However, they rushed me into getting ketamine right away and after a few days of getting the ketamine, I was back to being able to stand. It really is my miracle drug and so important that I have it especially if I am going to be having all this happens to me.
In addition, the doctors are going to try other treatments, medications, etc. to try to save my legs. My entire body has been discoloring worse than ever lately due to my neurological condition, which means lack of circulation. My legs especially have been blacker than ever and despite even elevating them, they really don’t resume normal coloration. The doctors are really worried that something is going to happen to them like amputation and therefore, they are trying to do whatever they can to ‘save’ them. One thing that they are going to do is possibly give me Viagra or Ectasy. Don’t laugh. Ectasy is an NMDA receptor and therefore, it might be extremely helpful because NMDA receptors like I have said in the past have the potential of reversing or stopping the progression of the disease. That is why ketamine works so well. They are thinking about using Viagra because it will hopefully increase the circulation. These are definitely 2 meds that I never thought I would be taking. However, they refused to start these meds out of the hospital because of all my complications. They said I need to be closely monitored.
However, ketamine is extremely dangerous and you have to be carefully monitored when you get it. That is another reason why I need to be placed in the ICU. I can only be in the ICU when I get it and in fact, not every ICU in the hospital will even do the ketamine. The doctor literally has to ask the ICU if they will do it and it is up to them if they will because the nurse has to feel “comfortable” because of how dangerous it is. It has to be done in the ICU because I need to be on a one-to-one and be so closely monitored.
The doctors are also going to try some other medications on me as well that are extremely risky to be tried as an outpatient. One such drug that they are going to try is low dose naltrexone. Low-dose naltrexone holds great promise for the millions of people worldwide with autoimmune diseases or central nervous system disorders or who face a deadly cancer, so perhaps it can help me too. By blocking opioid receptors, it is hoped that naltrexone is believed to produce a prolonged up-regulation of vital elements of the immune system by causing an increase in endorphin and enkephalin production. Many body tissues have receptors for these endorphins and enkephalins, including virtually every cell of the body’s immune system. In fact, when they did a study of giving this drug to people with autoimmune diseases, none failed to respond; all experienced a halt in progression of their illness. So perhaps it will help me too.
My brain tumor on my pituitary gland is also getting worse. The doctors want the neurosurgeons also to look into this as well because it is also wreaking havoc on my body. Since most doctors in other hospitals are hesitant to touch me and operate because of how risky and complicated I am, my doctor here at Hopkins wants the doctors here to look at it because they are used to highly complicated cases. Lately my cortisol level and prolactin level have been higher than ever and are way too high. These are all being caused because of the tumor and show that the tumor needs to be taken care of. So we will see what these doctors have to say. Never a dull moment.
When I saw the whole team of doctors today, the doctors also suggested giving me bisphosphonates as an infusion on a regular basis for my neurological disease. I get bisphosphonates once a year for my bones because I have severe osteoporosis thanks to this wonderful disease. However, even though this might be helpful to help treat my neurological disease and even my bones more because even though I do get it every year, my bones are still continuing to deteriorate. Even though this infusion makes me extremely sick too and I am still losing bone, the doctors are saying that if I wasn’t getting these infusions, I probably would be losing even more bone. So, I have been still continuing to get these annual infusions despite the fact that I am still losing bone because they believe that it would be a lot worse. Even though raising the dose and giving it more could possibly help maybe my bones and my neurological condition, it is completely out of the question because I suffer from osteonecrosis, which is a disease, caused by reduced blood flow to bones in the joints. In people with healthy bones, new bone is always replacing old bone. In osteonecrosis, the lack of blood causes the bone to break down faster than the body can make enough new bone. The bone starts to die and may break down.
The osteonecrosis has already gotten so bad in my jaw that it is protruding into my mouth. I have already had parts of my jaw removed because it is dead and there is plenty of more dead bone protruding and causing me even more pain. To have this pain and suffering on top of all my other pains and suffering from my neurological disease and gastroparesis, it really wreaks havoc on me. It is thought that perhaps the bisphosphonates might have contributed also to the osteonecrosis because they believe that since the jaw takes up so much absorption of the bisphosphonates, they think it killed the bone. So, if I were to increase the amount of infusions because of neurological disease, it would really harm me because of the osteonecrosis. It is like I am helped in one way and screwed in another in no matter what I do.
So that appears all that is happening. The next week or two are going to be extremely hectic because it is going to entail having a lot of talking to doctors and a lot of organization going on. As it is, we have been on constant phones with the doctors at Hopkins all day today even after we left them because they have been in constant touch with us. Even though we aren’t there, they are continuing to proceed with meetings about me and talk about me to make plans for my arrival and such. I never thought I would be able to say that I am going to know like doctors from every single specialty in a hospital. I can make like a whole directory of John Hopkins after all this.
The doctors were so impressed with my condition that they already warned me not to leave any records or pictures with anyone. Whenever I go to the doctor or to the hospital, I bring my entire medical records and pictures that document my disease so that they know exactly what goes on. After all, I am so complicated and something that isn’t seen everyday. I turn different colors, swell, shrink, etc. and to be quite honest I am a sight to see. That is I am on “show” when I go into the hospital. I constantly have everyone coming in to see me because I am a very rare case and something they don’t see everyday. It kinda bothers me that I am always on “show,” but if I can be of help to someone else and if they can learn off of me because they don’t see cases like me and you can’t really find me in a textbook, then I guess it is worth it. But the doctors want to make sure that no one takes my records or pictures as way of learning or having my disease to show others. You know?
So… I am heading home in the morning to enjoy the next two weeks. I am going to try to make them the best two weeks of my life because I never know what is going to happen when I come back. When we come back to Maryland, we are going to be staying in the same hotel. The staff at the hotel is amazing and they really treated us like family. They really made us feel comfortable.
Want to hear something funny? When we came back from dinner today, we went to go into our minibar in our room because we have been putting our sodas in there so we have them nice and cold to drink. We wanted to take our medications and therefore, when we went to get them out of the minibar, we ran into a huge problem. For some strange reason we couldn’t get the door open. Dad tried to pry open the door by various ways but no matter what he did, he couldn’t break open the door. Finally we called the front desk and they sent someone up. The stupid minibar locked itself. We were told it wouldn’t lock again and we thought we were safe. But now at 2 AM in the morning when I want something to drink… guess what? I am locked out again. Only my luck! In addition, the waiter made me some special Maryland crab dips and such to bring home to New York. We put them in the minibar as well in the meantime until we leave so that they can be kept cold. However, now that the minibar is again locked, I guess that is the end of that.
Well… I guess I am going to get going. I have had a big and exhaustive day. I really have a lot to think of and I am extremely scared. In fact, I never was so scared in my life. I don’t know how on earth I am going to be able to survive this. I am going to need all the support I can get.
I am also scared because August is my dad’s busiest month at work. I am afraid because with me being so sick and going to be in the hospital for so long, he won’t be able to work, which means that there won’t be any work really going out. Even though my mom will be remaining at home to watch the business, people are not going to want to wait around until my dad returns. Therefore, I fear the amount of work he is going to lose out on. I know how much of a hard time we have been having paying the bills, paying for treatment, etc. and I don’t know how are going to do this all… especially pay for this huge trip to Maryland… without the help of others because dad won’t have the business as an income during the time we are here in Maryland. We are having extreme trouble now paying the bills now and we have had to make cuts in my treatment with dad working, how are we going to pay for things when there is no work going out? If my father loses the business, I don’t know what I would do. I would never forgive myself if he lost the business because of me. I just know that during the next two weeks that he is going to work harder than ever (if that is at all possible) so that he can try to make up as much work as possible.
I just continue to hope and pray that everything will work out. I just hope and pray that not only will I make it until I am supposed to come back to Hopkins, but that others will continue to support me as well because it really does make a huge difference knowing that I have the support of others who care when battling this war. I also hope and pray that others spread the word of my website and bring attention to my illness so perhaps we can receive funds that can really help us out because it is so desperately needed. But… of course I am not going to count my chickens before they hatch because I have been let down too many times in the past.
In the meantime, I have been living on my ketamine at home. It has really been my miracle drug and made a difference. They recently raised my dose, but it still really stinks that it is so short-lived. I only get 45 minutes relief because the half-life is so short. But that 45 minutes is so worth it! Now I know why people call it “special K”!
Well… until next time….