Adventures with IgG

One of the reasons that I ended up here rather than at one of the other facilities was my Igg infusions. No one had experience with giving the Igg. They couldn't accept my Igg that I got from home because there were policies preventing patients from bringing medication into the facility from home. Because of the cost, they couldn't get it through pharmacy. So, even though I had the medication, everything needed to give an infusion, and even an infusion nurse who could come and give it, it wasn't possible to get it there. Here, they arranged it so that I would go up to Baystate to get my infusions once a week. While it seems that this should be an easy thing, things are never easy, and never without drama.

My appointment was at Baystate for 10 o'clock. The ambulance came at 9, and we were there in plenty of time. I had to take an ambulance since I was a patient going from one facility to another. We got to Baystate and after getting the run-around of where we were actually supposed to go, we got me settled. We got to the first floor, and were told to go to the 8th floor, then when we got there, they directed us back to the first floor, ect, ect. It was quite an ordeal. They get me settled on the stretch, but the stretcher is not in a bay with wall o2. There is only a portable tank underneath. Well this is fine, but of course, portable tanks go quite quickly when you're on 6L. Finally, they found a bay with an o2 hook-up. Why it was so difficult to find a place that had wall o2, I don't know. It would seem obtaining o2 in a hospital shouldn't be difficult. But then nothing is ever as it should be.

My appointment was for 10, but in actuality, they didn't even give me my pre-meds until 1. For some unknown reason the infusion took forever. At home the longest it's taken is 5 hours. I didn't finish until 9:30 (8 1/2 hours after starting).The day nurses left at ~6:30. So they had me moved upstairs to the observation unit. This is the unit that houses the patients that don't necessarily need to be admitted, but they want to watch them overnight to see how they are and make sure they don't need to be admitted. This is fine. I finish my infusion and they call for an ambulance.

As the ambulance comes, I need to use the restroom. The problem - the bathroom in the room isn't handicap accessible. I can't get a wheelchair in there. Well, ok. I then ask the nurse if I could use a handicap access bathroom on the floor. For sure, they have a visitor's bathroom and typically, those have to be handicap accessible. Though what happens if they have a handicapped patient who needs to use a wheelchair, I don't know. Well I am told that there isn't a handicap bathroom on the floor. What? No handicap bathroom? This is a hospital is it not? They didn't figure they might need a bathroom that was handicap accessbile and able to fit a wheelchair? The nurse then asks me if I want to use a bedpan. No, I don't wnat to use a bedpan. I want to use the bathroom! There's no reason for me to have to use a bedpan. I decide that I really have no choice but to wait until we get back to Mount Siani.

In the ambulance, my trach gets clogged with a mucous plug. There is nothing to clean it with and I can't breathe with it clogged. This isn't an emergency because being smart, I brought my extra trach just in case. You should always have an extra in case the trach falls out or whatever. So I open the extra trach, put the clean inner cannula in, and take out the clogged one. I don't want to just put the clogged one in the box, so the EMT offers me a glove. I put it inside the glove and ask him to put it in the box. I can clean it when I get back. No problem. Well, he didn't put it back in the box, but rather, he threw it out. Yes, he threw it out. It's not like that was important or needed. It only was part of my trach.

The drama continued as the EMTs got lost in Hartford. They didn't know how to get to Mount Sinai. Today this shouldn't be too big of a problem. Plug in the address into the GPS and voila. Nope, no GPS. Perhaps they should've referred to a map, or called dispatch to get directions, but they did none of these things. Instead, they pulled over and asked a random person on the street for directions. So, here we are in Hartford, at night, and they were pulling over to ask random people how to get to Mount Sinai. I have to wonder if they didn't secretly have a death wish. Thank goodness all was okay. Eventually they found it. I got back at 10 pm, 13 hours after I left. An infusion, which we expected to take up most of the day, but to be back by dinner, took all day and then some. This was supposed to be my "day off" from therapy. Though it wasn't much of a day off.

Rehab

After the third trach surgery, I woke up tremendously weak. It was as if my body had decided that enough is enough. I could not even get to the commode placed next to my bed without help. The original plan had been that I'd be in ICU to be monitored. I was kept for the weekend, and supposed to be discharged on Monday. When Monday came, I knew I wasn't ready to be discharged. I still needed extreme help with even simple tasks such as standing or getting to the commode next to the bed. The week before the second surgery, where the trach was downsized, was the worst. I was sent home though I shouldn't have been. I was weak and unprepared. The proper oxygen hadn't been set up. I didn't have trach supplies. I was basically confined to my room. This is not the life I had planned nor wanted. They promised that with a trach, my quality of life would improve. I would do the same things I did before the trach. Nothing could be further from the truth. Really I needed rehab to get stronger and learn how to live with a trach. When you first get oxygen, you have to learn how to live with it. You learn how to breathe. You get used to the tubing and carrying it with you. You learn about the machines that take over your house and how to fill portable bottles. You learn how to manage the oxygen when you go out. Well, it's similar when you get a trach. You must learn how to live with it.

It was said that I needed rehab. The problem is that once you leave the hospital, it is much harder to get in. When Monday came I told the discharge coordinator about my struggles to get into rehab. She was hopeful that she could get me in with no problems. Of course, with me, nothing is that easy. They got the insurance worked out, but the infusions were a problem. It was too expensive for them to pay for my Igg. I offered the use of mine since I get Igg supplies and meds for home infusions each month; it wouldn't be a problem to bring them in. However, there was a policy not allowing patients to bring in meds from home. It went on for days, trying to make it work. If we couldn't get it to work, then what do we do? I wasn't able to go home in my weakened state. I'd be unable to get in the house, nor get around the house, as would be necessary.

Finally, they tried Mount Sinai, which is an acute rehab facility. What is the difference? The reason the social worker had first tried to get me into Fox Hill was because it had pulmonary rehab. Mount Sinai does not. Fox Hill, however, is a sub-acute rehab facility, and like most sub-acute rehab facilities, it is part of a nursing home. Therapy is less intensive. A lot of the clientele is older people needing to gain strength especially after a fall such as a broken hip, or stroke. Mount Sinai on the other hand, is an acute rehab facility and is associated with Saint Francis Hospital. It is a hospital in itself with medical type units. Hospital rooms with jacks for oxygen set-up and hospital beds. They are able to take more medically complicated patients. Mainly, they treat patients recovering from an accident or brain trauma. They are capable of dealing with trachs and IVs. Thus, they were better suited for the care that I need. Rather than 1-2 hours of therapy a few days a week, here I will have therapy from 9 AM - 2 PM with a break for lunch, every day, six days a week. Patients get a day off from therapy. For most patients this is one day on the weekend. For me, since my ivig is nearly an all-day event, the day of my ivig will be my day off. I will have therapy both days on the weekend to make up for this.

Back to my story... I met with the case manager from Mount Sinai, and things seemed to be very positive. She expected me to be able to be admitted if not that day, the next; however, once again, I knew this too good to be true. The problem was my Igg infusions, again. They couldn't order it from pharmacy because the pharmacy didn't carry it in their formulary. I told them that I could bring in the supplies and meds needed; however, their nurses weren't qualified to give it. I could have Kathy, my infusion nurse come out and administer it, but this was against their policy. They couldn't have a nurse who wasn't part of their staff administer it. So once again, we were in the same position as we had been with Fox Hill. Going home was not an option. I got to the point that I said that if absolutely necessary, I would give up my infusions for the time I was in rehab. I knew it would be a maximum of 30 days because insurance would only cover for 30 days of rehab treatment. Though obviously not an ideal solution, going to rehab was more important. Without going to rehab I was unable to be home and return to my life. I was too weak to get in the house by myself. Once in the house, I couldn't walk, so getting myself to the bathroom and around the house wouldn't be possible. After much thought, they suggested having me going to Manchester Hospital to the infusion suite to get my infusion there. This would get around all of this bureaucracy and red tape. I would go to the hospital and get my infusion, just as I would if going to a doctor's appointment. I would get my infusion and then be able to return to rehab after. This seemed like an ideal solution. It was days of uncertainty and frustration. Everything is run by bureaucratic rules and regulations, which truly do nothing but prevent patients from getting the care they need and deserve. You have to jump through hoops and find ways around them just to get what you need. Finally, they were able to arrange it so that I would go up to Baystate to get my infusions once a week. To me this seems ridiculous since they now pay for an ambulance ride to and from there; they pay for the infusion suite and nursing care; and they pay for the Igg and medications. While had they done it here, they would have no additional charges. But all that really matters is that I can go to rehab and I can get my Igg infusions while there.

Though it took so long to get me here, I am proud of myself for being persistent, not just letting them bully me into submission, and not letting them send me home before I was ready, as they had done before. This is a dedicated rehab facility. The other patients are around my age. The oldest patient I have seen is ~60ish. It is very intense. I have ~5-6 hours of therapy 6 days per week. I have speech therapy, occupational therapy, and of course, physical therapy. All my therapists are extremely knowledgeable and encouraging. God works in mysterious ways.

Learning how to stand with PT Laura

Locks of Love

I began growing my hair a little over a year ago. At first, I started growing it because I thought that I was going to have to have a bone marrow transplant. I have been receiving IVIg treatment for my cvid for 5 years now. Typically replacement immunogloblulin therapy is sufficient to keep a patient with cvid healthy. Unfortunately, this has not been true for me. When I was first diagnosed and started receiving IVIg treatment, I received infusions once every 4 weeks. At first this helped. I was in the hospital less than prior to being diagnosed and starting IVIg treatment; however, I was still getting many infections, frequently requiring the need to be hospitalized. My doctor determined that we would increase the frequency of the infusions to every 3 weeks instead of every 4. Initially this helped to decrease the occurrence of infections, but once again, it did not stop it completely. After nearly another year of treatment, it was decided that perhaps a lower dose more frequently would more effectively manage the infections. The new treatment schedule had me receiving 10G IVIg weekly instead of 40G every 3 weeks. The idea was that a lower dose more frequently would eliminate the peaks and troughs, helping me maintain a more steady state of immulglobulins, giving me better protection from disease and infections. This is not how cvid is traditionally managed, but we were willing to try just about anything to get the infections under control. Initially, things seemed to improve greatly. I was able to go 8 - 10 weeks without being in the hospital for an infection. However, this was still not enough to keep me infection free. Not having any other options, my doctor decided to suggest the possibility of a bone marrow transplant. A bone marrow transplant would be a cure for my cvid.

CVID is the result of faulty bone marrow, which makes faulty B cells. In healthy bone marrow, B cells are made and they mature to the point that they can identify, attack, and kill potential pathogens and invaders. In people with cvid, the B cells never mature. Instead, they remain in a naive (baby) state, where they are unable to identify or kill pathogens. Since most people with cvid are able to be kept healthy by receiving Igg infusions, a bone marrow transplant is not even considered. Though a cure, a bone marrow transplant is a very risky procedure. To undergo a bone marrow transplant, the patient must first go through high doses of chemotherapy and radiation to completely destroy the patient's own bone marrow. Once a patient's own bone marrow is completely destroyed, they receive an infusion of a donor bone marrow. The donor bone marrow must be a perfect match. Even under the best of circumstances, it is nearly impossible (except in the case of an identical twin) to get an exact match. The problem is that without an exact match, the body identifies the donor marrow as foreign and attacks it, causing a variety of symptoms called GVD (Graft vs. Host). A person's body can reject the donor marrow completely if it is too dissimilar. Once the person receives the infusion of donor marrow, the donor marrow grows and matures. Once it is fully mature and functional, the person can go home. The donor marrow is free of the genetic mutations causing cvid, and thus the person no longer is considered to have cvid. It is because of the severe risks and complications that a bone marrow transplant is rarely done. The chemotherapy and radiation itself is extremely harsh on the body and a person can die from complications of these medications alone. The chemotherapy causes the person to lose all of their hair.

When the doctors first began discussing this as an option for me, I began growing my hair. I knew that losing my hair would be very traumatic. I love my hair. In fact, it is one of the only physical features of my body that I absolutely love about myself and would never change. Why? Well, first of all, my hair is a unique color. It is made of various colors and tints - browns, blonds, reds... It can't be described as one color, but is often referred to as honey blond. I have never altered my hair color in any way. I am proud of my God-given color. In the summer, the sun causes it to lighten and different highlights of various colors appear. My mom's hair dresser has spent countless hours trying to reproduce the color of my hair for my mom when she gets her hair colored each month. I am proud of my hair and its uniqueness. It is a symbol of who I am. Losing it, even if it is to have a bone marrow transplant and a cure to my disease, would be devastating. This is why I began growing my hair as soon as doctors began even thinking of such an option. If I truly needed a bone marrow transplant, I wanted to be able to cut my hair and turn it into a wig to be worn while I received chemotherapy.