I emphasized when making the appointment how ill Randi was and that we did not want to make another trip to Boston unless they felt certain there was something more they could offer Randi. After consulting with the infectious disease doctor to ensure this would not be a wasted trip Randi was scheduled to see two doctors, the infectiuos disease specialist and a surgeon. I made it clear we were not considering any further surgeries. Keeping in mind Randi did not do well during our last medical trip I was hesitant. None the less we both knew this was likely her last chance as all other options had run out. If Randi was willing so was I, there's nothing I wouldn't do for my children! Besides we welcomed any sort of natural medicine vs. man made. Maybe we could still get this infection under control after all. So off we were on yet one more medical trip. This trip would be more focused and only involve one day of appointments vs. a whole weeks worth.
Brooke needed to have a cortisol stress test the next time we were in Boston so that was set up as well. Brooke's baseline corisol level was found to be very low, same as discovered in Randi a few years earlier.
The infectious disease specialist was very open minded. Although she felt maggott wound therapy would not benefit Randi because her severe immune deficiencies were the root cause of these never ending infections vs. the actual woulnd itself she was willing to help us deal with Randi's chronic j-tube infections. Since Randi's immunologist had bowed out of the picture in regard to Randi's chronic infections, soley due to Randi's age, this was most welcomed. We discussed our only other option which was to have surgery to remove the j-tube and exclusively use intravenous access for all hydration, nutrition and meds. We had pondered this idea in the past but for a number of reasons felt it was not in Randi's best interest. The biggest factor being a number of the medications Randi relied on and were crucial to her well being were not available in an IV form. Probiotics being the biggest concern. The infectious disease doctor conferred that patients who were reliant on probiotics at high doses, as Randi was, did not have a favorable outcome when faced with this scenario. It was also noted during the office visit that Randi had a low grade fever. This was not a good sign, it meant something was brewing but I let the doctor know Randi had been running a low grade fever on/ off since the surgery in February. Randi had vomited a few times over the last several days as well but she was sure it was nothing more than her severe allergies causing post nasal drip. Due to her total gastroperisis any secretions swalloed caused Randi to vomit. In retrospect, however, there's no doubt that this was the beginning of the end.
If we were going to make the 5 hour plus medical trip we decided to tempt fate and go to Maine for a few days after the appointments. Maine is only an hour and a half from Boston. The girls and I so love the ocean. I knew it would be stretching the limits but I also felt it would likely be Randi's last chance to see the ocean. Little did I know just how true that was, these ended up literally being the last two days of Randi's life we could have got her to the ocean.
A few weeks earlier I had taken Randi to the movies. Since we were forced to go to a late afternoon showing I became ill while at the movies and had a hard time fulfilling my promise to take Randi to the movies that day. She knew it was hard on me but she said she was glad I brought her because she really needed to get out of the house. I kept that in mind and we agreed that if you're going to be ill, you're going to be ill and sometimes you may as well be somewhere that is pleasant and can take your mind off things for a while.
While in Maine both Randi and Brooke ended up spending the majority of their time in bed. Brooke's stomach pains and nausea were ever increasing as well. Having a room right on the ocean allowed me to have the windows and doors open so the girls could enjoy the ocean from their bed. Gazing out at the ocean from the door step where I sat I placed phone calls to Chicago. Randi had finally been referred by her endocrinologist for further workup of her hypercalciuria. I was arranging for specialized testing we would perform when we got home and send via mail to be annalyzed by specialists who dealt specifically with kdiney stones. We had just spent thousands of dollars consulting long distance with the best parathyroid specialists in this country in efforts to rule this out as the cause of Randi's high calcium levels which now were not only in her urine but blood as well.
We made short trips to get something to eat and down to the ocean. Brooke and I helped Randi walk to the ocean's edge so she could get her feet wet. We sat on the beach for a little while. Randi played in the sand a bit, something she loved to do. At that time she mentioned that her back pain was worsened but we both chalked it up to sitting upright for longer than Randi was used to. It wasn't uncommon for Randi to experience muscular cramping and spasming. Later that evening we took a walk down to the pier, Randi in her wheelchair. After about an hour Randi began to cry saying her back was hurting awful. Eventhough it was not like Randi to cry and complain, I'll admit I didn't think it was anything serious at first. I was upset because I wanted for us to enjoy our last night at the ocean. Randi did not sleep well throughout the night and by 3:00 am she was crying again. I took her temperature and it was high, something was very wrong. We had been through too many bad experiences that ended up being critical when ever Randi presented with a high fever. I woke Brooke and we both grabbed a quick shower, We hurriedly packed the car and headed back to Tufts Medical Center in Boston, where we'd been two days earlier. It was Thursday July 1st, just before July fourth weekend. The ER staff told us Randi would need to be seen in the adult section of the ER vs, pediatric where she is generally seen because of the holiday and staff shortage. We were told Randi would still have a pediatrician in charge of her care. One of the first things that they did was to order a new IV antibiotic but no one informed me of this. When the nurse came to administer the antibiotic I questioned this since I was already administering her two separate daily IV antibiotics. Its a good thing I did question things because they were ready to give Randi the exact antibiotic that almost killed her a little over a month earlier due to toxicity of her bone marrow. Randi lied there all day writhing in pain and only once did they give her anything more than what she arrived in the ER on as her usual pain med to ease her pain. All of our doctors were on vacation. The infectious disease doctor we had met two days earlier had her on call physicians examine Randi. Renal on call was also consulted. A CT without contrast was performed and it did show a number of small stones as usual but we were told there didn't appear to be any blockages. I was quite sure it was a kidney stone to blame. There were no other new symptoms that would lead us to believe otherwise. Blood cultures were also drawn. By 9:00 pm a nurse announced Randi would be admitted to the ICU for observation. I was told I would not be allowed to stay with Randi since she was going to the ICU. The only reason Randi was being placed in the ICU was because of her pain pump, which was hospital policy. From the time we arrived in the ER, I made it well known to all staff what our circumstances were. We were on our way home from a medical trip and visit to the ocean and therefore Brooke was with us and I could not leave Brooke alone as she required 24/7 care and supervision also. First of all, I was NEVER leaving Randi alone in a hospital! After all of our hospital experiences not a chance! Who would administer all of her meds.? No nursing staff in any hospital had ever been able to do this. There were so many reasons this was not an option. They suggested I find a room in a nearby hotel for Brooke, leave her there alone and I could sit in the hallway outside of the ICU. I was about to lose it! I asked to speak with the nurse manager, certain they would be willing to help us. I was greeted by two women, one claiming to be the nurse manager. They wasted no time in telling me nothing was going to change. I explained how all 3 of us were ill and this was unacceptable. Then they demanded I tell them which form of mitochondrial disease we have. I explained our family had a one of a kind form but they insisted I provide an exact name for our form. Then, In a very vindictive tone, one of them stated, "You're Dr. K's patients aren't you." She was referring to our mito specialist. It was a well known fact, which we've discussed with our mito specialist in the past numerous times, that admissions for adult mito patients was very complicated and hard to arrange. Rarely do I lose it but I'd been up half the night before, we'd been in the ER for more than twelve hours. I'd already made several trips to our car to drag Randi's supplies in for the admission. Randi was very ill and no one had done anything to help her all day. My adrenaline was pumping. I started screaming and packing everything up. I placed a call to our local mito specialist to ensure someone would be around over the holiday weekend to help us out before we headed for home. It wouldn't have mattered there was no way we were staying there but I didn't want to end up in our local ER upon returning home either. We faced a several hour ride home. I really did not know if I could do this. Never was I so tired while driving in my life! We had to stop to medicate frequently, it was so exhausting and Randi was so sick and in so much pain. One more nightmare situation all because of the lack of appropriate medical care for mito patients! I told everyone involved I'd rather take the chance of dying in a car accident than staying and being subjected to anymore of this kind of treatment.
The next day our local mito specialist increased Randi's pain medication.
I was busy getting ready for Brooke's birthday, which was July 5th but we were celebrating it on the 4th, when the phone rang. The caller ID said Tufts Medical Center. I knew what this meant before ever picking up the phone. It was someone from the lab letting me know Randi had a positive blood culture. They explained that the culture was very slow growing and contained thin gram negative rods they'd never seen before. The hope was that this was just a contaminated culture. We wouldn't know for sure until they could identify the organism after further incubation. Had the day we'd always dreaded finally come? Did Randi have sepsis? We had always been told by the girl's immunologists to never have any thing implanted in them due to their severe immune deficiencies for fear of sepsis. We were also told it was likely neither girl could ever survive sepsis for the same reason. Randi seemed to be holding her own if this truly was sepsis. I thought maybe since she was already on IV antibiotics she'd have a fighting chance. She got to enjoy her sisters birthday and the next day her overall condition worsened so I phoned our local mito specialist. Tufts still did not have the final organism identification. We were advised to obtain blood cultures locally in an effort to try and obtain a better culture for identification. Meanwhile Randi had another IV antibiotic added to her regimen and we decided to try and handle things at home for now. For the next few days Randi's condition waxed and waned. By July 10th it was clear we needed to go to the ER. I was pretty sure this was the end but I also wanted to give Randi every last chance possible. I decided to take Randi back to Albany Med for a number of reasons the biggest factor being Randi did not want to die in a hospital. Being critically ill, it would be much easier to get her home from a one and a half hour trip vs. a several hour trip. Randi's new infectious diseases doctor would be consulting with our local mito specialist and the last admission to Albany Med was not so bad.
Things had been resolved with Tufts after our last ER visit and I was assured we would never be treated like we were again. Besides writing a letter I spoke with patient relations as well as every one of our physicians at Tufts who in turn spoke with heads of the hospital. Therefore, I felt comfortable going back to Boston but under the current circumstances thought Albany Med. would be fine.
Albany Med.'s ER was on top of everything. Randi had the same ER nurse she'd seen a few months earlier. The ER doctor's consulted with our infectious disease specialist in Boston as well as our local mito specialist at Albany Med. The only problem that arose while in the ER was they didn't have one of Randi's IV antibiotics in the hospital. It was the only antibiotic left, at this point in time, that was effective for her j-tube infection. We could not risk this infection relapsing so I had to call home and have my son make a special trip down with Randi's week supply of IV antibiotics.
It was after midnight when Randi got to her hospital room. This was another all day adventure which I embarked upon on as soon as I awoke the previous day at 5:00 am.
It was Sunday so we never saw a doctor until approximately 2:00 pm. The doctor marched into the room followed by two other individuals and confronted me to my face. To my knowledge, I had never met any of these people before so I wasn't sure what this was all about. First she asked if I was aware of Albany Med's medication policy. I said, "Randi was admitted approximately two months earlier and there weren't any issues. I acknowledge it is customary for the hospital's pharmacy to check over medications brought in from home and I am fine with that. Further more I've always administer my daughter's medications while in every hospital she's ever been in.simply because it is impossible for nursing staff to do so." I wish I could have relinquished this task to nursing staff. The reality was there were so many medications and the frequency with which they were given made this impossible for nursing staff. Also, it was imperative that Randi received her medications when they were due. To that the doctor replied, "Well, somehow that was slipped past me because I would not have allowed that." Next, I was asked if I was aware of Albany Meds. visitation times. I was shocked, what was this all about? Randi was gravely ill, I was just trying to hold it together and these people whom I've never done anything to are attacking me, why? I fired back with, "You don't want to go there, do you, do you really?" I pressed her for a response as I was not taking this! Are you telling me I can't stay with my daughter, are you, are you? The doctor's reply was a hesitant no you can't then they all quickly exited the room. Randi was gravely ill, she was developmentally disabled, legally blind, needed assistance with everything, had extremely comlex and complicated problems that only I knew about! I didn't know what to do. I placed a call to Randi's local mito specialist at Albany Med. While I was on the phone the doctor returned to Randi's room. I asked her what her name was so I could convey that information to our specialist. The doctor asked me who I was speaking with and I told her Randi's mito specialist's RN. I tried to explain how Randi's mito specialist worked at Albany Med. and he could vouch for Randi's needs and that he should be consulted. The doctor stated," If she has a doctor here at Albany Med where the hell is he?" I didn't know if I should stay or take Randi and go to Boston. I still felt it unwise to travel such a distance with Randi so ill. We'd already invested 2 days at Albany Med. and hadn't gotten anywhere. If we went to Boston we'd have to start all over going through their ER . The nurse manager came back in and told me they decided I could stay with Randi and that we should just forget about the whole incident. When she left the room Randi pipes up and says, "Yeah right, they think I am dumb and I don't understand what they are saying and doing, its not right."
Randi's medications were all taken from us and we were guaranteed that there would be no more than a 15 minute window from the time her meds. were due until she received them. The nurse manager said I might be adding to Randi's illness by over medicating her. There was absolutely no reason for her to make this accusation. Every single medication Randi took was prescribed by her speicalists. In fact there were other medications that were prescibed that we refused to use or were unable financially to obtain. After being put through the accusations of Meunchaunsen's once, I vowed to never, ever, let anybody treat me like that again. Things settled down a little until the nurse came with Randi's next dose of medications a short time later. First the nurse didn't have all of Randi's scheduled medications. Randi was on two separate forms of potassium, each given for a different reason. One was for her severe hypokalemia the other for her kidney stones. The nurse said the doctor ordered the potassium stopped since Randi would be receiving it via IV. I demanded the nurse page the doctor to straighten this out. We could no longer get meds., nutrition or hydration in via Randi's j-tube. Any attempt at using her j-tube brought upon hours of retching. This was a major problem because not all medications came in an IV form. Randi and I agreed to keep trying to get the the few crucial medications in her via her J-tube that could not be administered IV. It was heartbreaking to keep putting her through this agony every few hours. If we couldn't get these medications in we knew it would lead to further serious problems for Randi at a time she desperately needed each and every one of these medications. Then the nurse drops the anti nausea medication on the floor, the only small pill Randi took orally, since it could not be crushed, dissolved or administerd in any other way. She picks it up and hands it to me telling me I can give it to Randi now. I took it and threw it in the garbage. I said, "Are you crazy! What's wrong with you? My daughter has sepsis already and you want me to give her something off the floor." She immediately dove into the garbage can after the med. stating, "You can't do that its a controlled substance." She leaves the room only to have the nurse manager come in telling me they are writing me up for harassing the nurse. WOW!!! Does anyone take responsibility for their actions! At this point I demanded Randi's medications be returned to us. I'd had it, I really thought we needed to go to Boston. And, once again I go over every thing in my head weighing everything involved. I look at Randi and I ask her what she wants to do. She is as upset and hurt as I am. I placed another call to our local mito specialist to see if they can please intervene because I could not tolerate any more and was at my wits end!
Later that night the doctor came to me after she had time to review Randi's medical history and problem list which I provided her. She amitted I knew what I was talking about. Great, but it was already too late. Initially, nobody would listen to me about the need for Randi to receive large daily doses of potassium replacement and the need to draw these levels to assess. Now her potassium was critical and they would have to run bag after bag of it IV. The problem was she had so many other bags of nutrition, and IV antibiotics to run that there literally was not enough time in the day and night despite running them non stop to infuse all of them. Then I was asked to chose what she would receive and when. The next day a nurse brought Randi's medications into the room and told me I could have them back. They were catching on!
Everyday blood cultures were drawn, at least twice daily, and with every culture one more different rare organism was confirmed.
Making matters worse the infectious disease doctor at Albany Med., who was consulting with our new infectious disease doctor in Boston, was the jerk that we met a few months earlier during Randi's previuos hospitalization. I asked that he not be involved in randi's care and we were told he had to be because he was Albany's best. If Randi could not even remain infection free on IV antibiotics for the past 2 years, how was she to come off them? There was no reasoning or even rational conversing with this man. We'd run into doctors like this in the past and eventually most of them had come to realize although my children never fit the norm their unique presentations and responses were real. Unfortunately, we didn't have the time to prove this to this man nor would I have wanted to waste my time doing so.
Then Randi and I brought to everyone's attention that the arm her picc line was in was very swollen and hard. Ulrasound documented 2 blood clots. The line was pulled and the tip cultured. The three organisms that had been identified to date, were on the tip of the line. With no other options we had to place another line immediately. Randi reiterated that when the infected line was placed during her last admission at Albany Med. she felt the staff was acting reckless and haphassard. She was adament about this. I was not allowed to go into the room with her but she told me as soon as I joined her afterward. I had always accompanied Randi all other times when her lines were placed.
The next day brought more bad news. I was being asked for permission to give Randi a blood transfusion, something we had always feared given Randi's immune dyfunction and the known reactions to IV IGG. We decided to wait a day and see if her red blood cell count might come back up on its own. Unfortunately, that wasn't the case and we forged ahead with the transfusion. Luckily, Randi didn't experience any obvious problems. That was good because she required anothertransfusion the next day as well. I didn't know how Randi had hung in there this long. She was so sick and weak. We were forced to stop j-tube feeds on the Saturday night Randi was admitted. It had taken four days for any IV nutrition to be ordered and started. No nutrition of any sort for four days in a critically ill patient. I also asked for Randi to be given hydrocortisone due to her adrenal insufficiency. The doctor's response was, " If she were in the ICU I would." How much sicker did Randi need to be before someone realized how gravely ill she was. I had always feared and predicted that someday Randi and or Brooke would die from an infection because they do not present normally with infections and by the time someone acknowledged this and figured it out it would be too late.
By Thursday we were ready to leave it was clear there was nothing more that they could do for Randi in the hospital that we couldn't do for her at home where she'd be much more comfortable. Each blood culture result grew a new organism. I knew Randi's immune system was incapable of fighting one blood borne organism let alone several. Her immune system had finally failed it was best to take her home. Although the infectious disease doctor thought her heart had to be harboring the organisms in order for the antibiotics not to be working, an usltrasound did not indicate this. I knew it was simply the end, what we had always knew would happen someday. Nobody took the severity of Randi's illness seriously until now. Even at this point Randi and I still didn't feel everyone was on the same page. Randi and I asked for hospice but were told we didn't need it. Both Randi and I were unsure of what was to come and thought we'd feel better with hospice on board. Instead of receiving compassinate care and understanding in her greatest time of crisis and need she had to endure a nightmare during her last hospitalization. We could accept there was nothing further that could be done. What we could not accept was the lack of appropriate medical care and support!!!
Early Friday morning I placed a call to our infectious disease doctor in Boston to discuss everything. She was upfront with me and said she was going on vacation for a week and she hoped we would talk when she returned but that she doubted Randi would make it that long. She explained that one of the organisms Randi had, of the several, was in itslef almost always fatal. Just that morning we discoverd all of Randi's extremeities to be rock hard and swollen. She said that was a bad sign and to be expected as part of what sepsis with acenitobacter causes. After placing a call to our local mito specialist letting him know we needed for him to write orders for at home so we could leave, I sat and talked to Randi. I let her know what our Boston specialist had said. Randi then told me she already knew she was dying because her uncle Mike had come to her, telling her everything was going to be alright. She hadn't shared this with me until now although she'd known before we ever went to the hospital. Randi had a connection with the other side and was very intrigued by such. My brother, Michael, had passed away at the age of 27. Randi was nine years old at the time. Shortly after his passing Randi saw his face and he told her that everything was going to be alright. Prior to a family members passing, Randi would see Mike. He would tell her that everything was going to be alright. I started to cry and by then our mito specialsit had arrived with his RN. They quickly took care of everything we needed so we could leave. I was overwhelmed. I was so used to Randi's old schedule that I had performed over and over for the past three years. Nothing was the same now that she exclusively used IV's for everything. We could do this, afterall we'd provided Randi with 24/7 RN level care at home for the past 2 years basically all alone. Randi's brother would help me with the nightly IV infusions so I could catch a little rest. Neither Randi or I had gotten mcuh more than a few hours sleep each night the entire week she was admitted. Brooke was also helpful she gave Randi her bed baths and was good company for Randi. I was familiar with a lot of different scenarios as to how Randi's final days may play out. I could only hope that a situation would not arise that would force us into a hospital situation that neither of us ever wanted to go through again.
Before leaving, the doctor who was assigned to Randi that had been so vindictive initially towards us gave Randi a hug and wished us the best. It seemed she finally understood, most doctor's eventually come to understand but as usual, it came at Randi's expense and this time it was too late!
Once we were discharged, as was customary, I asked Randi if I could get her something special to taste. She asked for cheese pizza but I couldn't find any quickly so she had to settle for a milkshake. Unfortunately, she was so sick that even attempting to taste brought about the horrid wretching she'd experienced over the past week. I just had to get her home as quickly as possible. Upon arriving home we got her into bed immediately. We both needed to catch up on a lot of sleep. That was the best thing Randi could do, sleep. Family and friends were called and told that Randi was brought home to die. For the next four days she only awoke for 2-3 hours each day and even then she would go in and out of conciuosness. This was a good thing given her condition. During her waking hours family and friends stopped by to visit. My mom and dad's 50th wedding anniversary was in a few months and we had talked about getting a family picture. We are fortunate to have a friend who is a photographer who was able to come to our home on short notice to capture a family photos for us.
A few days after being home I received a frantic phone call form Albany Meds lab. It was a physician demanding I drop everything and immediately bring Randi back to Albany Med. because she had a positive blood culture. I tried to explain to her that we had taken Randi home to die and that she already had sepsis with multiple organisms as she continued to argue with me. You would think that this would be noted in her chart somewhere and also they'd have our doctor's number for contact in such situations. I was still drawing labs and blood cultures on a daily basis and bringing them to our local hospital. I received a phone call form that lab also with yet another positive blood borne infection. This time it was a systemic yeast infection.
Randi's last four days were anyhting but restful. Her heart rate, blood pressure and breathing were all abnormally high. It was as if she were running a marathon non-stop. I knew she could not keep up at this pace for long.
By the fourth day, Sunday July 25th, it was clear Randi was very aggitated. she had been unable to sleep much during the night. I drew her labs and headed to the hospital. My son had been delivering the labs to the hospital for the past few days. I did not want to leave Randi knowing she could need my help or pass anytime.
I feared if something happened while I was away someone might panic and call 911, but this morning I just had to get away. Driving home from the hospital I said to myself, why am I continuing to torture Randi with these daily blood cultures. I made the decision that there would be no more. Brooke and I tried to comfort Randi with various things throughout the day but nothing helped she just held her abdomen writhed in pain, moaning and groaning. This was the fourth day Randi struggled to keep up with the out of control pace of her heart , blood pressure and breathing. I prayed to God to take Randi and spare her anymore pain and suffering. Our local mito specialist called in the afternoon to let me know about Randi's critical lab values. He said he'd increase Randi's pain medication the next day. I told him I didn't think Randi would still be here by then. I began Randi's nightly medication ritual a little early just to do something for her. She got up onto her side in bed and was swishing her mouth with water after taking all of her inhalers. My mom was over to visit and asked Randi again, "Is there anything I can get for you or do for you. Randi's reply, "I still want to see Justin." I went to dump out the basin and by the time I returned, two minutes later, Randi had gone back down in bed and was shaking uncontrollably. Randi couldn't tell us if she was cold which was doubtful as it was oppressively hot outside. However, she had drawn her comforter around her. I calmly sat beside her and held her. What ever was happening I felt sure was the beginning of the end. Brooke joined us and the children's other grandmother, who had just called to check on Randi's condition came right away to see Randi. Since their grandmother was very upset she felt it was best to leave. Brooke, Drew, Blackberry and I all sat with Randi. We told her we loved her and stroked her hair. Drew lifted blackberry up onto Randi's bed and he gave Randi a couple of big sloppy kisses. A few minutes later Randi said she needed to get onto her back then she said she needed to sit up higher in bed . Drew and I helped boost.her up. Next she said she was going to be sick so I reached for her basin and that was it. My sweet angel baby was on her way to heaven! Randi let us know that everything was alright by the big perfect smile that came over her face. We called my mom and dad over and we all just sat peacefully with Randi for quite a while. Watching and waiting for a loved one to pass is not easy but I know we made the right decision to have Randi at home during this difficult time. I did not want an autopsy or for Randi to be embalmed. I didn't want anyone doing anything to Randi ever again! She'd been through far too much in her lifetime!