Back to surgery we go....

Well, looks like I'm going back to surgery tomorrow. :(

I have been having difficulty breathing since I had the new trach put in on Tuesday. The doctors just told me this was normal and that I just had to get used to the change in size in the trach. But going from an 8 to a 6 isn't that much of a difference and certainly shouldn't cause this much difficulty breathing. I just feel like my airway is restricted. But I'm new to all this and so maybe they are right and it's just I have to get used to it.

Then last night I coughed up a lot of blood with big clots. The problem is that one of the clots was so big that it wouldn't pass through the inner cannula and almost clogged the trach itself. My aunt was asleep and I nearly freaked out because I don't know how to suction myself yet and if it had gotten clogged, there would be nothing we could do except call 911 and hope they get here real fast because if the trach is clogged, I have no airway. Yeah, a little anxiety provoking. Thankfully I was able to cough it up after much work of trying to force as much air as possible out of my lungs to dislodge it and get it out. Pretty gross. Anyway, even so, I felt like something was obstructing my airway. With the previous trach when I felt like that it was something stuck in the inner cannula. I'd take the inner cannula out and clean it and everything was ok. But when I took out the inner cannula, not only was it not dirty, but it didn't help the feeling. I still felt like something was obstructing my airway. Well, thankfully the VNA came today. I had her show me how to suction myself, but when we went to put down the suction catheter, it would only go a little ways and then it wouldn't go any further. It was like it hit a wall or something was preventing it from being able to pass. I thought initially it was something I was doing wrong, but the nurse had the same problem, she couldn't suction me. It certainly wouldn't go down far enough to suction - no cough reflex or anything. So she called the doctor because apparently this isn't normal and maybe the reason I feel like I can't breathe and that something is obstructing my airway is not because I'm crazy or that it is anxiety, but I truly have something obstructing my airway. One idea is that it may be inflammation. The fear with this is that if we don't get it under control, it could obstruct my entire airway and that would be very bad... The nurse also told him about the amount of blood I'm still coughing up. Apparently this is also not normal, which is what I was told by the ER doctor.

So off to surgery we go again tomorrow. They will put me under like they did on Tuesday and he will go down and take a looksy to see what's going on down there and what is causing both the bleeding and the obstruction. Need to make sure I can be suctioned. That is very important as this is my airway. And for the second time in 3 days I'm NPO after midnight. The problem isn't regarding food. It's not like I'll waste away to nothingness by not eating, but not drinking sucks. I get so thirsty. Why is it that the words NPO make you hungry. You could be so sick and not want to eat at all; as soon as they say the words NPO, you are starving and want to eat.

I'm not too keen on the having to go back under anesthesia again since my last reaction was so stellar and though I can't see why it would cause any pain as they aren't cutting or suturing this time, I can probably expect, knowing me, to be in pain when I wake up. I mean last time they weren't cutting either, just putting in some sutures to make sure the excess hole closed properly, and I woke up in massive amounts of pain. If there is inflammation, don't know what he'll have to do other than give me meds to decrease it. If there is bleeding, guess he might have to cauterize something. Good thing my surgeon is a thoracic and trauma surgeon as well. He told me this kind of jokingly when I first met him to put in the trach. He went down the list of possible complications and then said, but good thing if anything on this list happens I am a thoracic surgeon as well, so I can fix it. lol. I laughed, never thought I might actually need that and be glad he was. Not saying that I will. I'm not trying to over dramatize things. But just in case he needs to fix a bleed, he can do it. That makes me feel somewhat better. Though Rockville is not exactly the hospital to be having major surgery, but I guess you don't always plan these things.

I've been considering once the trach is in place and we are over these hurdles to switch my care to one of the bigger hospitals - probably Hartford Hospital, but I can't exactly switch surgeons and care when all these things are happening. I need to stick to the same surgeon. And though it would have been best to have the initial surgery at Hartford Hospital, when they tell you you need a trach, you don't exactly say ok, but you need to transfer me to a different hospital first where I feel they are more equipped to deal with emergencies. It is a pretty emergent procedure and you want to be where your doctors are and they are on staff at the hospital so that if something happens you are being treated by them. Unfortunately that means Rockville or Manchester, and I will not go to Manchester unless I have a death wish.

The major frustration with all of this is why don't doctors ever listen to the patient. I said I felt like I couldn't breathe. Told them I didn't feel the amount of bleeding was normal. But I was told this was just anxiety. I didn't really know what I was talking about. I mean, it's just my airway and my breathing. Obviously the doctors know more than me regarding my breathing and what feels right and what doesn't. I've only been living in this body for 28+ years. But having the initials M.D. after their name must make them experts on all bodies and what is normal and not. It's a rare complication so obviously I can't be having it. I just have a rare immune disorder and we're doing this procedure in hopes of treating another rare disorder and this procedure for this cause has only been done 3 times world wide, but I can't be having any type of rare complications. I tell you, I should play lotto. Then the VNA nurse tells them what's going on and all of a sudden I'm not quite so crazy anymore and maybe, just maybe, I should be taken seriously. As I said, it's only my body, but I don't know anything about it.

Trach Surgery

Tuesday I went in to have my trach downsized from an 8 to a 6. Supposed to be a simple thing, but I'm a true zebra and whatever is supposed to be simple is not. First of all, I now understand HoJo's anxiety when she had her bone marrow biopsy and she couldn't have her passy meir valve. With me since I can't yet use the passy meir valve, I have a writing board and pen. You write on the board (kind of like etch-a-scetch) and press a button and it erases it. Anyway this is my only means of communicating currently. I obviously couldn't have this in surgery and this made me very nervous because I had no way of communicating with anyone at all. At one time, just before they put me out, they had the trach mask on but it wasn't on right or something was blocking the trach, or something, but I couldn't breathe right. Well the problem was my arms and hands were all wrapped in for surgery so I didn't fall off the table and so I couldn't adjust it myself, and I couldn't get the nurses' attention or the anesthesiologist. I started freaking out and finally they figured out what was up and fixed it, but for a moment it was quite scary.

I never deal with anesthesia well, not even since birth. My first surgery was at 7 months. They gave me anesthesia then and they give it to me now and it never fails, I wake up crying every time. After the initial trach surgery I was extremely disoriented. I didn't know that surgery was over or had even begun. I was on the vent and because I was so disoriented I fought the machine terribly. I finally calmed down once I figured out where I was and what was going on. This time I asked the nurses to make sure they told me where I was and that surgery was over so that I wouldn't be disoriented after. This helped. But I was told that there wouldn't be any pain. Well I knew this meant I would have some pain, but nothing is pain free and doctors don't know what they are talking about. But oh, my God, I was in extreme pain. 2mg dilaudid, didn't touch it. Finally after another 2mg it was starting to be under control. He had to use a lot of stitches because apparently there was a lot of bleeding. So that is one source of pain. I also had a lot of swelling and so initially couldn't swallow even water. They gave me sips of water and I ended up choking. I also woke up with horrible coughing. I kept coughing and coughing, these horrible whole body coughs where you can't catch your breath at the end. You feel like you're coughing your insides out. This of course hurt like hell and I was coughing up massive amounts of blood - totally not cool. Every breath I took, I ended up having the horrible coughing fits. The only thing to avoid them was to not breathe, and that wasn't exactly a legitimate option. So, yeah, after surgery was not fun.

I had so much difficulty with my breathing and it was getting to be 5 o'clock, when the nurses want to go home. Supposedly they had no way to directly admit me so they sent me to the ER. Well the ER was packed, but thankfully I got right in, being a day surgery patient and all and having difficulty breathing. But once in the room, that was a different story. I had the same doctor I had last week when I had to go in with the bleeding. He is a real asshole. Thinks he knows everything, but in truth knows nothing. He's new and honestly that just makes everything worse. They didn't even call my pulmonologist. Gave me some pain medication and some ativan and that's it. Didn't listen to my breathing. Didn't pay attention to the fact that my sats were bouncing from 79% to 90%. Yes, the 90% is ok, but the 79% not so much. We found out I had a fever before surgery of 100.8. This is extremely high for me because 1) my normal temp is 96.5 2) I never run a fever even when sick, so if I do, it's quite alarming. Based on this, we knew something was amiss. After surgery my temp had gone up to 101.6. Definitely not good. The doctor didn't even draw blood. He did do a chest xray, which came back clean, but then a chest xray is only going to show pneumonia, not much else. He determined that this was just anxiety and I just should go home. So home I went. I didn't actually get home until midnight. It was a long day and they never resolved my difficulty breathing.

Things are Never Easy

I was discharged from the hospital a week after having the trach placed. So I was discharged on last Thursday. Since then, life has been a collasal horrible disaster. They basically discharged me without any proper discharge plan. They were supposed to downsize the trach to a 6 so that I could use the passy meir valve and talk. Remember this was supposed to make the quality of my life better. Well, the surgeon decided that the trach was too new and he did not want to downsize the trach for another week. Well we will skip ahead in time to today when we called and guess what.... the doctor is on vacation this week. So is my trach going to be downsized so that I can use the passy meir valve this week? That would be a giant no because the doctor is on vacation. Can I scream? Oh wait, I have no voice! I can't even do that. And while I was told that this was a simple office procedure, guess what I find out. It is day surgery at the very least. They will bring me in, change the trach and it may be a few hours or I may need to stay over. Let's count on me needing to stay over because that is how my life is. And honestly I just want to be sure I can use the passy meir valve and talk. Because this not being able to communicate sucks and is just not fair. Just sayin'

So back to the story. The company that previously handled all my o2 needs does not do trach care or anything with trachs. Ok, understandable. Trachs take special consideration and are more complicated than plain just o2 needs. So one would think before being discharged from the hospital they would set me up with the o2 needs and humidification that I need with a trach. Also, one would think they would provide me with what I need for trach care. For those who don't know about trachs, they obviously have to be cleaned and taken care of. Well, none of this was taken care of. I was sent home without any humidification for my o2. This caused me to get very dry and that caused other problems. Also, I was sent home with 2 trach cleaning care kits when discharged. Since PromptCare, my previous o2 company could not provide my needs, it was set up that Apria would take over. Well Apria never got the proper authorization, scripts with specific o2 needs, ect. and so they never set things up. Anyway.... VNA came out Friday and were appalled that I basically had nothing - o2 wasn't set up and had no trach care kits. It was also determined that I should've been sent to rehab not directly home since I have not learned how to live with a trach, nor care for myself with a trach, and after having the trach put in, I'm sufficiently weak and really can't do much. So, yes, I need rehab. We were told to go back to the hospital and they would admit me and arrange for rehab. Well I never actually made it into the ER. I sat in the waiting room for several hours while the charge nurse made some phone calls and tried to arrange for the o2 needs with Apria. We all know that it is near impossible to be admitted for rehab on a Friday afternoon at 2pm. So we were assured that Apria was going to come and by 7pm when they hadn't, we were back on the phone trying to get the humidification I needed to get through the weekend, since nothing was going to be done until Monday. In the end, we went to the ER and picked up some humidification bottles to attach to the liquid tanks. We rolled one liquid tank into my room and attached the humidification bottle attached to the trach mask. This gives me the ability to move about a foot and a half. Basically I'm stuck in my room. So much for making my life more mobile.

On to Saturday. The VNA nurse comes again. At this point I have used all the trach care kits given to me because my trach keeps getting clogged with huge blood clots that require me to take the trach tube out and clean it because I can't breath through these clots. The nurse calls the dr that put it in (yes the doctor that is currently on vacation) and he says to go to the ER. So off to the ER we go. Also decide to mention to the ER that despite 80mg of lasix my feet are so swollen with severe edema that I can't walk and nothing fits on my feet. Oh, but does the ER want to do anything? nope. They give us a few more supplies and tell us that after talking to Dr. Constantino (the dr that sent us to the ER) that coughing up blood is normal for a new trach. (then whey did he send us to the ER?) They did do a chest xray and that is all fine. So we left with one more trach kit, some ampules of saline to squirt in my trach, and a few more supplies  to get through the weekend. Apria still has not come with o2 or supplies.

So we get through Sunday. I sleep and watch tv because there isn't much else I can do. It is now Monday. We try to get me into rehab. The VNA and my pcp are trying to work together. There is a rehab facility in town that does pulmonary rehab that would be perfect. Guess what? Medicaid won't cover skilled nursing care. haha. Life is such a collasal joke. I can't get into rehab because it isn't covered by insurance. Did I mention that Apria still hasn't come. The pulmonologist is supposed to be signing and faxing over orders so they can deliver what I need, but yet it still hasn't been done. We have been making phone calls all day. My pcp's suggestion was to go back to the ER, to which we siad no because they aren't even going to treat me but rather they will just send me home again, so what is the point of sitting there for hours. Now it is 4 o'clock and despite countless calls made by my mom and VNA since I have no voice, still no change. VNA is trying to get the pulmo to direct admit me and finally do the job they should've done originally. Yes, some of my doctors suck. Some of them are great and are trying their best to move mountains and get things done. My VNA nurse is an angel and really couldn't do more than she is.

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.

The Truth about Vitamins

When you have a chronic illness, some people who don't understand the illness offer suggestions of non-traditional or holistic ways of curing you. One big thing that people suggest is taking vitamins. I have been told been by some people that I don't need to take all the meds I do, but that I just need to take more vitamins. Take some vitamins and you'll be cured. People today often are part of a vitamin craze. They think vitamins will cure any ill they may have or could get. Some take more vitamins than I take pills. They have to get the super pill containers to hold them all. They take several handfuls of vitamins several times a day. In this, they believe more is better; however, more is not always better.  Taking too many vitamins can be harmful and even toxic to the body.

Vitamins are what is known as micronutrients. They are organic compounds that are required as nutrients in small amounts. They must be obtained by the diet and are not synthesized by the body. There are thirteen vitamins, which fall into one of two classes: water-soluble vitamins and fat-soluble vitamins. There are four fat-soluble vitamins (vitamin A, vitamin D, vitamin E, and vitamin K).

Water soluble vitamins, as the name suggests, dissolve easily in water and are excreted from the body as urine. There are nine water-soluble vitamins (the B vitamins and vitamin C). Fat-soluble vitamins are absorbed in the intestinal tract and stored in lipids (fat cells). Since they are stored in fat cells in the body, they are more likely to accumulate. Therefore, too much of fat-soluble vitamins can be toxic to the body and lead to hypervitaminosis. Hypervitaminosis is due to a vitamin overdose. This usually occurs by high supplement intake, not dietary sources.

Water-Soluble Vitamins:
Vitamin B1 (Thiamine)        

• Dosage:  1.2mg          
• Function:  coenzyme in metabolism of sugars and amino acids
• Natural Sources:  yeast, oatmeal, flax,  sunflower seeds, kale, brown rice, rye, asparagus, cauliflower, potatoes, oranges, pork, chicken, beef, liver
• Deficiency:  Beriberi, Wernicke-Korsakoff syndrome (occur in alcoholics)
• Over-dose: (5,000-10,000mg) headache, irritability, rapid pulse, weakness

Vitamin B2 (Riboflavin)

• Dosage:  1.3mg
• Function:  energy metabolism; metabolism of fats, keytones, carbohydrates, proteins; maintain mucous membrane, skin, cornea of eye, and nerve sheaths
• Natural Sources:  milk, cheese, leafy green vegetables, liver, kidney, legumes, tomatoes, yeast, mushrooms, almonds
• Deficiency:  skin disorders, light sensitivity, inflammation of soft tissue around nose and mouth

Vitamin B3 (Niacin)

• Dosage:  16.0mg
• Function:  precursor to NAD+/NADH and NADP+/NADPH (energy metabolism); DNA repair; production of steroid hormones in adrenal glands
• Natural Sources:  protein-rich foods (meat, fish, yeast, eggs, milk, legumes, potatoes, peanuts)
• Deficiency:  Pellagra
• Over-dose:  (35mg) liver damage, flushing, tingling, itching, headache, nausea, diarrhea, ulcers

Vitamin B5 (Pantothenic Acid)

• Dosage: 5.0mg
• Function:  synthesize co-enzyme A; synthesize and metabolize protein, carbohydrates, and fats
• Natural Sources: (in nearly all foods) whole-grain cereals, legumes, eggs, meat, royal jelly, avacado, yogurt
• Deficiency:  parenthesia, hypoglycemia
• Over-dose: (extremely rare 10g/day) diarrhea, nausea, heartburn

Vitamin B6 (Pyrodoxine)

• Dosage:  1.3 - 1.7mg
• Function:  sodium/potassium regulation; red blood cell production; cofactor in neurotransmitter production (serotonin, dopamine, epinephrine, norepinephrine
• Natural Sources:  liver, meat, brown rice, fish, butter, wheat germ, whole-grain cereals, soybeans
• Deficiency:  (rare - due to alcoholism) anemia, peripheral neuropathy
• Over-dose:  (100mg) impairment of proprioception, permenent nerve damage, impaired walking, numbness, tingling, poor sense of touch

Vitamin B7 (Biotin)

• Dosage:  30.0 micrograms
• Function:  metabolism of fatty acids; role in gluconeogenesis
• Natural Sources:  egg yolk (without egg white), liver, vegetables, peanuts
• Deficiency:  alopecia, conjunctivits, dermatitis

Vitamin B9 (Follic Acid)

• Dosage:  400 micrograms
• Function:  synthesize, repair, and methylate DNA; aid in cell division and growth; necessary
• Natural Sources:  leafy vegetables, legumes, eggs yolk, yeast, whole-grain cereals, sunflower seeds, liver, kidney
• Deficiency:  anemia, poor growth, neural tube defects in developing fetus in pregnancy, peripheral neuropathy, mental confusion, heart palpitations, irritability, behavior disorders
• Over-dose:  (1,000 micrograms) mask symptoms of vitamin B12 deficiency

Vitamin B12

• Dosage:  2.4 micrograms
•  Function:  regulation and synthesis of DNA; fatty acid synthesis; energy production; formation of blood; normal function of brain and nervous system
• Natural Sources:  animal sources (fish, shellfish, meat, eggs, poultry, milk
• Deficiency:  pernicious anemia

Vitamin C (Ascorbic Acid)

• Dosage:  90.0mg
• Function:  antioxidant; formation of collagen; improve iron absorption and resistance to infection
• Natural Sources:  vegetables (broccoli, green and red peppers, collard greens, brussel sprouts, cauliflower; fruits (lemon, pineapple, strawberries, citrus fruits)
• Deficiency: scurvy

Fat-Soluble Vitamins
Vitamin A (Retinol, Beta carotine)

• Dosage:  900 micrograms
• Function:  vision; normal skin
• Natural Sources:  liver, carrots, broccoli, sweet potatoes, butter, kale, spinach, pumpkin, collad greens, milk, cheese, peas, eggs, apricots, mango, papaya
• Deficiency:  impaired vision, blindness, impaired immune function, hyperkarotosis
• Over-dose:  (3,000 micrograms) nausea, irritability, vomitiing, diarrhea, blurred vision, headache, insomnia, hair loss, muscle and abdominal pain, bone fractures, loss of appetite

Vitamin D

• Dosage:  5.0 - 10.0 micrograms (requires sunlight to be effective)
• Function:  aid in immune function; regulate calcium concentration in blood; mineralization, remodeling, growth of bone; regulate proliferation, differentiation, apoptosis of cells; regulate neuromuscular function; reduce inflammation
• Natural Sources:  fatty fish, eggs, meat
• Deficiency:  rickets, osteomalacia

Vitamin E

• Dosage:  15mg
• Function:  protect red blood cells; prevent destruction of vitamin A and C; protect skin; heal scars
• Natural Sources:  seeds, nuts, green leafy vegetables, tomatoes, pumpkin, sweet potatoes, mango, asparagus, broccoli, papaya, olives, avocado
• Deficiency: (rare) hemolytic anemia in infants, myopathy, peripheral neuropathy, impaired immune response
• Over-dose:  (1,000mg) increase risk of congestive heart failure

Vitamin K

• Dosage:  120 micrograms
• Function:  protein synthesis; blood coagulation;
• Natural Sources:  green leafy vegetables (spinach, swiss chard, broccoli, kale, cauliflower, cabbage, brussel sprouts, avocado, kiwi
• Deficiency:  (rare) bleeding diathesis, bleeding, anemia, bruising, osteoporosis, coronary artery disease
• Over-dose:  increase coagulation, allergic reaction, hemolytic anemia, cytotoxicity in liver cells

As you can see, vitamins are important to the health and normal function of the body. Most vitamins can be obtained from natural food sources; however, in cases in which deficiencies occur, vitamin supplements are needed. People often take vitamin supplements thinking that the more they take, the healthier they will be. This is not true. There is only so much the body is capable of absorbing and using. If too much of a water-soluble vitamin is ingested, it will be excreted as urine. This has minor effects on the body and basically a person is just paying for very expensive urine, as the cost of some vitamins is quite substantial. If too much of a fat-soluble vitamin is ingested, it will be stored in the fat and can become toxic. Like any medication, vitamin supplements also have side effects. Taking vitamin supplements such as a daily multivitamin can help improve your health, however, it is important to remember that too much of anything can be harmful.

Winter Fun

I woke up this morning to 23 inches of snow. I have lived in the Northeast all my life so snow is nothing new to me, but I have never seen this much snow. As I looked outside, I was reminded of all the fun I had as a kid. Waking up to snow meant a snow day; it meant making snowmen, going sledding, and hot chocolate when I came in. I felt so frustrated because I couldn't do these things. Not because I was too old, you are never too old, but because of the o2 and my health.

Usually I hate the snow because it means cold. It is inconvenient and you have to shovel, but this morning I desperately wanted to go out and build a snowman. It was probably because after my visit yesterday and feeling like this disease was progressing and having no answers, feeling like it was taking so much from me and having no way to stop it. Going outside in the snow would be one big FU. Yes, I was on o2, but so what. I could go out with my 100 ft. tubing. I knew it'd be long enough. So that is exactly what I did. I bundled myself up just as my mom had done when I was a kid - thick sweatpants, thick socks, ski pants, second pair of dad's wool socks over ski pants, shirt, sweatshirt, jacket, gloves under jacket sleeves, scarf, and hat. I made sure I was well protected.

Bundled up and ready to go outside

The snow was nearly up to my hip. I walked a little ways on the deck, feeling like an astronaut. I was tethered by my o2 tubing just as they are to the spaceship, bundled up as if in a spacesuit. Al the world around me was white with snow flying through the air, more snow than I've ever seen at one time. It was as if I had entered another world.

My original plan had been to build my snowman in the backyard, but after walking only a few steps, I realized I would not be able to walk that far. My breathing would not allow for it. Even after the few steps I had taken, I was short of breath and had to take a break. As I sat down in the snow to catch my breath, I decided to build my snowman in the middle of the deck, right where I was. The snow was not snowman snow. It was soft and powdery and did not lend itself to molding in any way. Typically you would take a handful of snow and mold it into a ball. Then you would continue to mold it until it got large enough to build it on the ground, rolling it and making the ball bigger and bigger until it is the size you want. You do this three times, each ball slightly smaller than the previous one. Then you place the balls on top of one another, the smallest for the head.

Molding my snowman

This snow would not do this. It was so powdery that by trying to make a ball, it just sifted apart through my fingers, but I was determined. I NEEDED to make this snowman. He represented more than just a snowman. He represented my need to fight this disease and not give up. My mother always told me that there is more than one way to skin a cat. So I mounded the snow up in a hill, patting it and packing it as I went. When it was large enough, I started to mold it. I dug out areas to that instead of a mound of snow, it looked like two rounded balls. I didn't have the energy to make three. When finally done, I added two sticks for arms, two Oreos for eyes, and a carrot stick for a nose. I then decked him out in a mask, o2 tubing, and nebulizer. I was so proud and happy. I had accomplished what I had wanted and didn't let my illness or the o2 get in my way.

Masked snowman

Snowman with o2

Snowman doing his nebs

Pulmonology Update #3

I had my follow-up at Mass General with Dr. Ginns yesterday. I had been really nervous prior to this appointment because I was scheduled to do a 6-minute walk and pfts. The pfts didn't make me nervous as I had done them a million times before. This is not to say I was looking forward to them. Pfts are often very triggering to my lungs. So much so that often I nearly end up in the ER and once was admitted to the ICU; however, it was the 6-minute walk that scared me. I had never done a 6-minute walk before.

The purpose of the 6-minute walk is to determine your exercise capacity. The objective is to walk as far as possible in 6-minutes. My fears about the test were that I didn't know whether I would be allowed to wear my o2 during the test or if they would try to do the test without. I wear o2 continuously, and without it my o2 saturations drop significantly, especially on exertion. In fact, I have to increase my liter flow when up and walking. Typically, I wear 5L at rest and 6L when walking.

Recently, someone asked me what would happen if I didn't wear the O2. This is a legitimate question for someone who doesn't need o2 and has no breathing problems. This is what I explained to them. First I would become short off breath. I would become so short of breath that talking would be difficult. Then I would have difficulty remembering things and understanding things. If a person were talking to me, I would have difficulty understanding what they were saying or asking. I would also have difficulty interpreting what they were saying, making it hard to give an appropriate response. Then I would become sluggish and lethargic. Finally, I would pass out. It is very uncomfortable to not be able to breathe and have your brain and body deprived of oxygen. Thus, the idea of doing the 6-minute walk without o2 scared me.The 6-minute walk can be done with or without o2. The only reason they would do it without would be to see how much your o2 levels dropped on exertion.

The test was no where near as bad as I feared. In fact, as tests go, it was pretty easy. I was taken to a long hallway, in which a line of tape had been placed on the floor, I was allowed to use my o2 and at the typical settings that I would use at home. Before the start of the test, my o2, bp, and heart rate were measured. I was also asked to rate my difficulty breathing. I then walked as far as po0ssible (up and down the taped line) in the 6-minutes. I was told to go at my own pace and was allowed to stop if needed. I was able to walk 343 feet. I did have to stop a few times to catch my breath.

After the 6-minute walk, I did pfts. I only did spriometry because the technician did not want me to overexert myself, especially after just having completed the 6-minute walk. The good news is that my lung function has not changed since my pfts in October. I am still at 20%.

After all the test had been performed, I saw Dr. Ginns for a follow-up. He went over my previous test results (CT-scan  and barium swallow) as well as the tests performed that day. Unfortunately, Dr. Ginns had no answers for me. The barium swallow was negative for acid reflux and the chest CT was relatively normal. So he has no explanation for why my pulmonary function is so severely decreased. Since he has no answers as to why my lung function is so poor, he has no ideas as to how to improve it or fix it. As hard as it was to hear this, it was harder to see how this affected him. I could see how badly he wanted to be able to provide me answers and how it pained him to say that he didn't have any. On hearing this I felt like I was going to cry. I have lost so much to this disease and it's hard not to be discouraged. I want answers, but more than that I want someone to tell me that they can help. What do you do when the best doctors don't know how to help? Yet in these moments I try to be strong for me and for those around me. It is hard not to be scared though. Yet I have faith and hope.