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| Our Maple Tree |
In front of me, I have my letters from my appointments with Dr. ANS. I've only had three in person visits because we have done all of our communication over the phone and through email. It's so striking to look at the letter from 2012 and compare it to the letter from this latest appointment. The notes are very personal, but they are also profoundly descriptive of my life with this illness. I have chosen to share large parts of them because I think the risk outweighs the costs.
I want others with this illness to know they are not alone, I want my friends and family to have a better sense of what the illness is like, and I want someone reading this to recognize the illness as what they might have or a friend might have.
In today's blog post I will share the letter from 2012, which is powerful and hard to read because it reminds me what a difficult time this was for me. Dr. ANS's letters are like no other I have from medical appointments and capture so vividly the physical, emotional and spiritual aspects of his patient.
I realize this is a long post, but I also think it is of value in the sense that it articulates things that I haven't been able to articulate for a very long time. Many times people ask me to write health updates on my blog and I get overwhelmed by the enormity of it all, by the sadness of it all, by the trauma of it, by all of the information. I think this letter is the best summary that could be written of my experience, treatments and life. Next week, I will share the letter from the most recent appointment, which is a breath of fresh air from what the past few years have been for me.
As I lie in bed tonight, readying to publish this post, I think about my day today which included a short trip to the mall to buy a cute sweater dress to wear with leggings. Yes, Mom took me, pushed the Rolls and helped with the clothes, but I didn't even used to be able to GO shopping for clothes. She did it all.
This evening, I went out to the restaurant where we had our reunion and met up with a classmate for dinner. (I'm working trying to have regular quarterly meet-ups for local classmates).
I'm amazed and in awe that life could change this way.
Blessings,
Emily
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| Our 40 Year Old Tree |
February 29, 2012
Emily returned on February 28, 2012 for a re-evaluation
of her chronic fatigue syndrome, fibromyalgia, postural tachycardia syndrome, anxiety,
prior tick-borne infection, and intolerance of milk and gluten.
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| Fall Colors |
Her daily function has been slowly but progressively worse in the last few years. She spends most of the time each day in her house, and activity of any sort is capable of aggravating her symptoms. A big challenge to improved quality of life is her inability to reliably sit up long enough to engage in any meaningful activity without aggravating her autonomic symptoms. She is able to write a blog on line once weekly, but does her writing in a reclined position. She watches TV in a recliner or in an adjustable bed. She eats one meal sitting up with her legs under her, and eats dinner in a recliner. Her mother helps with many of the activities of daily life, such as washing her hair, as raising her arms overhead causes weakness and autonomic symptoms. She is out of bed to walk within the house, and can walk 20 yards for the mail daily, but uses a wheelchair for trips out of the house. She can manage one errand or appointment in her main window of improved energy between 1:30 and 3PM, but this is her maximal activity for a given day. If she exceeds her limit of activity, she gets increased post-exertional malaise. Reading more than a brief amount increases her fatigue, as does talking with friends or having visitors to the house.
Her main symptoms now, as they have been throughout the illness, are profound fatigue, intolerance of more than a few minutes of upright posture, episodic symptoms of autonomic activation (agitation, heart pounding, air hunger, weakness, diaphoresis) after neck movements and upright posture. Her neck always seems tight; the neck discomfort is less bothersome in the mornings, and gets worse as the day goes on. Pressure against the occipital area bothers her, and causes her to feel as if she needs to move, but she does not report headaches. She reports urinary frequency, generalized myalgias, and insomnia. GI symptoms including bloating, cramping, and nausea have been improved after a cholecystectomy in 2005. She continues on a milk-free and gluten-free diet.
In the past, once she awakened at night, she would be kept up at night by urinary frequency, bladder spasms, and abdominal spasms. She gets fewer spasms after instituting a new program of lymphatic drainage, myofascial release, and cranio-sacral therapy. Starting Cortef and stopping DDAVP seem to have reduced the urinary frequency.
Her evening routine involves several hours of attempting to wind down. She falls asleep an hour after taking Klonopin and Ambien. She is up briefly to urinate and take medications at 8AM, then returns to bed until 11:30 AM. Sleep is unrefreshing. She does not snore, and a 2008 sleep study showed no evidence of obstructive sleep apnea. She naps for 1.5 hours in the afternoon.
She develops air hunger, pallor, and sweating after dinner. She does not report much lightheadedness, unless she arises quickly. For her autonomic dysfunction, she remains on Corgard 10 mg daily, midodrine 10-12.5 mg every 3.5 hours up to 5 times daily. With past symptomatic exacerbations, she has responded on occasion to 2 liters of warmed normal saline IV. She wears compression garments including 30-40 mm Hg Sigvaris stockings and spanx abdominal compression garments.
Other medications have helped control some of the symptoms related to autonomic nervous system excess (Lexapro 10 mg qD and clonazepam 0.5-1 mg daily have helped with agitation, lightheadedness, and anxiety). The chronic pain has responded in part to Lyrica 75 mg daily. Among the medications she has tried, the following in the last 5 years have been either ineffective or poorly tolerated for the autonomic, sleep, and pain symptoms: propranolol (weak, more fatigued, more pale, chest pain), gabapentin (worse), topiramate (worse), Marinol/dronabinol (worse), Dexedrine (more agitated), Cymbalta (worse fatigue), Effexor (agitation), Doxepin (ineffective). Effexor XR helped, but she was more agitated at higher doses.
In 2005, given the general lack of improvement after 7 years of illness, and how sick she was after the cholecystectomy, along with the degree of apparent neural irritability (pain, increased symptoms with neck movement, neural tension abnormalities identified on physical therapy screening exams, in the absence of anatomic abnormalities on MRI of the brain and cervical spine), we speculated that she might have a chronic infection as a perpetuating factor. She received a year of IV Rocephin, along with prolonged trials of IM Bicillin, malarone, Plaquenil, rifampin, and azithromycin. She is now on Cortef 7.5 mg daily and a number of oral supplements supervised by Dr. Complex.
Her day still consists of quiet activity in the house, much of the time reclining, although she attempts to introduce as much activity as possible to avoid further deconditioning. Along with a loyal group of friends, her mother is a main source of physical and emotional support. Emily has been in counseling since November with Evelyn, which has helped her mood. Anxiety is worse of late. She feels as if she is a constant state of anxiety, for reasons that are unclear to her. She reports feeling easily overwhelmed by tasks she has to complete with limited energy, and is often more overwhelmed at night when lying down and thinking about the day. Stresses include the issues of how to live meaningfully when this impaired, and how much to push versus rest, how much to accept the chronic illness versus continue trying different medical remedies.
Investigations recently include a repeat cervical MRI on 08/29/11, which showed no cervical stenosis, despite a strong family history of cervical stenosis. Her mother, for example, underwent a cervical fusion in May 2011. The lumbar MRI showed no evidence of a tethered cord, although she had some mild disc bulging at L4-5 and L5-S1, and mild epidural lipomatosis. Her lab work has generally showed a normal ESR (12 in 08/2011), normal CBC with differential, normal comprehensive metabolic panel and thyroid function (TSH 1.79), and a mildly elevated high sensitivity CRP in the 6.36-13.6 range (normal 0-5 mg/L).
Medications: Ambien CR12.5 qHS, mg; Allegra 180 mg qD PRN; Astelin 2 sprays to each nostril qd – bid PRN; Clonazepam 0.5 mg strength, 1 - 2 tablets qD; Cortef 5 mg AM and 2.5 mg at lunch; Corgard 10 mg qD; Lexapro 10 mg qD; Lidoderm patches (lidocaine patch 5%) 1 qD; Ranitidine 150 mg qHS; Lyrica 25 mg AM and 50 mg qHS; Midodrine 10-12.5 mg every 3 ½ hours up to 5 x daily; Junel OCP continuously, 365 active pills/yr; Fluocinonide 0.05% cream PRN; Nystatin and triamcinolone acetonide cream PRN; Tylenol 8 hr., 2 tabs qHS; Imitrex injectable PRN; Emend PRN; Relpax 40mg PRN; Metoclopramide PRN; Zofran ODT PRN; Colace PRN.
Supplements: Saccharomyces boulardii BID; OptiMag 125 2 tabs BID; Taurine 500 mg BID; HistoXym 1 qD; Cortisol manager 3 tabs qHS; Pregnenolone 30 mg qD; Probiotic 30 billion cfu qD; Folate supplements; vitamin B 12 10,000 mcg sublingual TID; vitamin D3 2000 IU, 2 qD; Alpha-lipoic acid BID; CoQ10 50 mg BID; Repair Guard 2 tabs BID; Pantothenic acid 2000 mg BID; Multivitamin BID; DHEA 10 mg qD; A-L complex qD; Os-Cal 500 +D BID.
Deep tendon reflexes were 2+ and symmetrical. Her Hoffman sign is negative. Pupils are equal and reactive. The optic disc margins are sharp. She has a positive Romberg test, falling backwards within seconds.
Her trapezius muscles are tight. Neck extension provokes immediate lightheadedness and a woozy feeling. With arms overhead, she develops diaphoresis, facial flushing, and slower verbal responses within 15 seconds. I could not detect a radial pulse on the right side with her arm overhead. Ankle dorsiflexion is limited, with 90-95 degrees of dorsiflexion on the left, and 95 degrees on the right. Passive straight leg raise elicits stretch at 45 degrees, associated with forceful heart beats. Upper limb tension testing with a median nerve bias lacks 20 degrees of arm extension on the right, but has normal range of motion on the left.
IMPRESSIONS:
Emily has a disabling chronic illness characterized by a number of symptoms that suggest a state of heightened sympathetic nervous system tone, including forceful heart beats, air hunger, diaphoresis, anxiety, and fatigue. Her profound disability persists despite valiant efforts on her part, and a concerted effort to control symptoms using a variety of medications and other treatments. I am struck today by several features of the illness:
· any neck movements, especially neck extension, consistently increase her autonomic symptoms
· she has some features of thoracic outlet syndrome, with obliteration of her radial pulse on the right with arms overhead, and increased autonomic symptoms with arms overhead
· her neurological examination is notable for a positive Romberg test
· her physical therapy screening testing continues to show provocation of her typical symptoms with passive straight leg raise or arms overhead
Her neck seems to be a trigger to symptoms, and I would like to investigate this further with weight bearing flexion and extension MRI images to look at cervical instability. I will forward a requisition for these studies.
I think she would also benefit from evaluation by a specialist in thoracic outlet syndrome. I will defer to Dr. Listener on which thoracic or vascular surgeons in Pennsylvania might be able to help sort this out.
Among the medications that might be helpful to her in the future, L-DOPS (Droxidopa), a norepinephrine precursor molecule, is close to being brought to market, pending FDA hearings this spring. It has been used extensively in Japan, and is thought to be safe and well tolerated. A second medication with some potential to help improve pain is naltrexone, given in low doses. There is some preliminary work suggesting this can be helpful for fibromyalgia pain if given in a short-acting form, in low doses of 1.5-4.5 mg at night. She will think about whether and when she might want to try this.
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