Spinal Muscular Atrophy (SMA) Werdnig-Hoffman's Disease
Jean Uelman, Antigua, Guatamala.
In 1996 Jean came to the beautiful city of Antiqua, Guatemala to die. At 50, she had lived well beyond the expectations of one born with the rare genetic disorder known as Werdnig-Hoffman's Disease also known as Spinal Muscular Atrophy. Her two siblings, both born with the disease, died before the age of four. Jean had more than just survived. She went beyond the struggle to breathe and swallow and manage within the constraints of a wheelchair. Jean had gone on to receive a Ph.D. in Psychology and had established and maintained a successful practice throughout most of her adult life. Now, with the disease so advanced and the pain so relentless, it seemed that the time had come to prepare for the inevitable.
In February of 1997 a friend brought news that a physician in Guatemala City was conducting a study involving patients with Rheumatoid Arthritis. The friend went on to say that the subjects in the study had experienced dramatic reductions in pain. Jean didn't have arthritis but she did have severe pain. So with little more than a remote possibility to reduce her level of sciatic pain, Jean and her friend set out to meet this doctor and find out more about what the patients had taken. Dr. Alejandro Nitsch proved to be more than a compassionate man. In spite of his limited supply of Cytolog, he gave Jean a bottle of it, and with it a full measure of prayer.
Following are the exact entries made over a period of six weeks that Jean made in her personal journal upon starting Cytolog. It is important to understand that Spinal Muscular Atrophy is a disease characterized by decline, not improvement, and that she had lived with and learned to accommodate that decline for almost 50 years. Out of respect for Jean's efforts to record a careful account of her experience, her log is presented in its entirety. It provides in real time, an unusual and very intimate perspective on her results.
Notes from Jean Uelman. (March 19, 1997):
Feb. 27,/ Start Cytolog at measurement 5 (1 teaspoon). Chronic sciatic pain (right leg), chronic low back irritation and recent shoulder-neck pain stop (not to return). Other discomfort from sitting in wheelchair remains.
Feb.28-Mar. 3,/ Increased mild tingling throughout body. Startled response to loud noise. Caregivers notice it is easier to move me - I'm not so limp. More energy. In my tilting wheelchair for 10 hours on Saturday with no sciatic pain.
Mar. 4,/ Physical therapy and acupuncture treatment with Dr. Caceres. He reports more body warmth and muscles feel less "stringy". The night tingling becomes so intense that it is difficult to sleep. (Night and some day tingling are this intense for five days and sleep is difficult).
Mar. 5,/ Clear increase in muscle usage. Able to use hands and arms more. Able to produce stronger motion in water bed. Flexing of muscles in legs visible. Notice that I no longer awaken in the morning with pain (for past ten years it has been the awareness of pain which woke me up each morning).
Mar. 6-10,/ Very small increase in muscle usage noted each day. I feel like I sit straighter. Ability to sit up on toilet without the use of a pillow (lost about 10 years ago) returns. Am able to do craft activities that I have lost over the three years. More stamina-need fewer rest periods. Breathing seems deeper. Coughing/clearing throat easier. Friends notice I am warmer to the touch and my coloring is not so blotchy. Able to move feet a little. More sensitive to start of pain from poor circulation when I sit in wheelchair and able to flex muscles to increase circulation and head of increased pain.
Mar. 11./ Physical therapy and acupuncture treatment with Dr. Caceres. He reports even more body warmth and muscles feel like they have a little more mass. Treatment less painful. That night dramatic increase in heat produced by my body.
Mar. 12-14,/ Increase Cytolog from 5 to 7.5. Continue with greater body heat (especially at night) -warmth feels good and body state more relaxed. Increase in alertness. Rest times in day now only needed when I haven't slept well at night. Have gained 5 pounds in past month (bad). Notice my skin is less dry and less "baggy". Ability to do crafts still improving and am able to keep at it longer before muscle movement begins to fatigue. Notice that over past two weeks my metabolism seems to have improved so that I am having a bowel movement daily rather than every three days (also more hungry). Have been taking NO MORE pain medication, not even a Tylenol before I go to bed.
Mar. 14,/ Increase Cytolog to 5 twice per day (6:00 A.M. and sometime between 5-7 P.M.) Neck seems stronger. Good day, but at night too alert. Lots of muscle tension (distracting but not painful). Sleep difficult.
Mar. 15,/ In morning sleepy but feeling good. Feel "stronger." While sitting on toilet chair (feet don't touch floor), able to produce an obvious rocking motion in the trunk of my body and am able to start my legs in a small swinging motion (right leg side to side-left leg front to back). Also able to move arms more (I lost such motion about 5 years ago). Tingling which had continued but become less noticeable, increases in intensity again (in morning). Night time, begin with mild stomach flu (others in house have it). Feel weaker, but not as weak as I felt before the Cytolog. Body heat and tingling stop for about six hours. When heat starts again, I feel better, like I have rounded the corner of the flu. Joint pain associated with the flu also stops when heat begins. Tingling does not begin. Bad night with nightmares that my body is in sections that must be connected.
Mar 16,/ Slept late. Still feel a little weaker, but heat and mild tingling present. Nausea present until midday. After nausea goes, feelings of strength seem to return. Disturbed by nightmares of night before and decide to do some hypnosis to reintegrate my body image. My body is different now and it seems to be having an effect on my psyche. Feel better after the hypnosis and realize the feelings of body tension relate to muscle tone that I did not have before. Feel good in the afternoon - energetic. Am using my wrists more - don't need supports under them when I type on the computer with my two pencils (still need supports for my arms). Can take lots of deep breaths and no longer feel like my trunk is collapsing inward.
Mar 12,/ Tried old, non-tilt wheelchair for a few hours. After half an hour intense pain begins. Not sciatic, but still horrible; pain meds don't even help. After out of chair, legs tingle intensely and pain stops immediately. In afternoon use tilting wheelchair with no pain.
Mar 18,/ Treatment with Dr. Caceres. Again treatment hurts less. He says I am warmer, and my neck is smoother. Veins in my arms are easier to see, and he wonders if my blood volume is increasing. Blood pressure: 110/80. Turning on my left side and working the right side also went better. After, the tingling more intense again. Am having some muscle spasms in my feet... Feels strange to have muscle activity there. Lots of tingling in my hands during the night.
Mar 19,/ Feel good in morning. Able to sit in non-tilt chair, using added pillow underneath, for an hour with minimal pain, but then chair breaks, nearly causing a serious accident on ramp. My heart beats rapidly after that, causing tingling in my hands again. Later when on computer, am definitely typing faster with my two pencils. Able to open heavy books (couldn't for 4-5 years) and use a scissors I could not squeeze before.
Mar 20-22,/ Still lots of energy. Am now accustomed to tingling and heat because only feel them when I think about them. Thus, am sleeping well at night. Other strange sensations, however: there's a pressure from the inside, where muscles would be, as if something is expanding or pushing outward. It's unpleasant as if it should hurt, but there is no pain. And my brain seems to be commanding certain movements I couldn't do before. I still can't do them but I start to before I see that I can't (Examples: reaching for something that's too far away or moving my leg because it's uncomfortable). So strange, I'm used to being in tune with myself (even though the tune has always been a slow waltz!). Am doing a lot, but nothing I can point to as an improvement over three days ago. Last night I dreamt that I could feed myself again - nice...Lost two of the five pounds I gained.
Mar 23-26,/ Went to Itzapa. Used old chair and did okay combined with pain med. Able to tolerate 3-4 hours (Before Cytolog, could only tolerate 1 hour). Went in pool and able to do so much more. Now can move right arm in arc (in January could move just a little), can move left arm some now, and can swivel hips. Can't unbend legs yet. Had treatment with Caceres on Monday before going into pool Tuesday able to feed myself! (Lost that 14 years ago.) Must prop arms with rolled towel, but am able to manipulate fork. Don't get tired either! Realize movement is returning in the exact same order it was lost. Next should come ability to raise hands (use arms). Also brushing teeth with arms propped. Before the Cytolog, when brushing teeth, both hands had to be held up by the wrists and even then it was difficult. Got bad sunburn in Iztapa - Could Cytolog make me more susceptible to sunburn ?
Mar 27-29,/ Feeding myself all meals and even popcorn, which means coordinating fingers and moving closer to mouth. Can't pick up glass for drinks; remember that I used to use a straw - should try that again. Having muscle discomfort, especially in my arms. I think it relates to using the muscles more. Am using my left arm more to support activities (propping, it too); for a long time it was as if I didn't have a left hand because it was so useless. Sleeping well. Sliding my right arm better when lying down, move it without using my fingers so much to help it along. This batch of Cytolog does not taste so strong and seems diluted. I wonder if they are different because of processing. Changes seem to be coming in patterns: more dramatic after a session with Caceres (he feels it is the acupuncture which pushes the Cytolog along) but noticeable improvement about every three days.
Mar. 30-Apr 1,/ Yesterday increased Cytolog to 12.5 spread over three times per day. Can now raise my right lower arm into the air when laying down. It must have a push from my fingers, wobbles some, and veers in: but I can bring it to my shoulder by itself! Treatment with Caceres this morning. He says that my joints are looser, more moveable. We're going to try some acupuncture daily for four days.
Mar 30 - Apr. 1 (cont.) My skin is healing nicely after that terrible sunburn and I'm finally starting to peel. Sleeping so well, I figured out, in part because I am now moving a little in my sleep! Able to lift my head a little and slide it, shift my frame just a little, and shrug my shoulders, Would be wonderful if I was able to turn in bed a little again. We'll see...
Apr. 2-5,/ Did acupuncture the four days with no particular improvement except that I am getting a little reaction at the needle insert. Progress continues although it is not as dramatic. Bringing my right hand up better (not so wobbly), and now am moving my arm and lifting it a little from the propping. Much more strength in my neck. Overall body strength improving. Was another bad cold/flu in the house and again I didn't catch it (No longer must live in fear of a sneeze!). Sleeping well except that I'm still getting some aching in my muscles as I use them more (good sign!).
Apr. 6-9,/ More movement in left arm/hand. Able to slide it 8 inches to a new position when lying down. Joints even more flexible - opening mouth wider and turning head better. Caregiver noticing my arms are even stronger in exercises. Using lower prop when feeding self (pulling in my hand still more). Night after treatment with Caceres, again I felt a strong "expanding" sensation from within. Activity seems to help. (This is a change from when activity tended just to tire me. Maybe now I have enough of what ever the Cytolog gives me to sustain me in activity so the muscles can get the benefit of the stimulation). Progress continues more steadily now although slower. Just to be improving at all is fantastic. New bottle of Cytolog; this one taste stronger.
Apr. 10-13,/ Now improvement seems to center on trunk. Am hitting the headrest of my chair differently so am a little taller. My stomach muscles seem to be kicking in (my abdomen is firmer) which should help my breathing. In beginning March, I had tried to learn bead sewing, but it was just too hard and I gave up in frustration; tried it again and now I can do it with no problem. Not only am I stronger, but using two hands is a great help! Had stomach flu again, but taking Cytolog seemed to calm my stomach. Slept a lot that day.
Apr. 14-16,/ Stomach still not feeling right. Continued progress with trunk of body. No longer feel like I'm a sack of corn meal. My body now has more structure and holds itself "together" better. I sit much better in the bathtub, with much better balance. Also, when in the chair, beginning to raise lower right arm (bending at elbow). Only goes up less than an inch, but it is a beginning. Caceres very pleased at treatment - says there is even more muscle mass, and he was able to do a more strenuous workout. In one week jaw opens tiny bit more at the dentist - 19mm to 20mm.
Related Information Sites on SMA
SMA, Ability not the Disability
SMA, Adult Onset, The Jennifer Trust
SMA, Florida Medical Network
SMA, The Jennifer Trust
SMA, Vanderbilt Medical Center