Myotonic Dystrophy and OT

Myotonic dystrophy affects approximately 1 in 8,000 people worldwide. The incidence of the two types of myotonic dystrophy varies among geographic and ethnic populations. In most populations, Type 1, the congenital form, appears to be more common than Type 2, a milder childhood-onset form.  Myotonic dystrophy can occur in patients of any age.  Both forms of the disease display an autosomal dominant pattern of inheritance.  Type 1 patients often have myotonia (muscles that won't relax), daytime sleepiness, dysphagia (difficulty swallowing), disabling weakness in their extremities and severe cognitive problems. Type 2 patients commonly have muscle pain, stiffness, fatigue, daytime sleepiness and the development of lower extremity weakness.

Physical and Occupational Therapists as well as Speech Language Pathologists (SLP) are usually brought in to help manage the patient's condition.  Either in the hospital, an acute rehabilitation centre or at home PT and OT assist the patient to participate in strengthening and aerobic training at a moderate intensity to increase muscle strength, promote heart and lung function, as well as maintain mobility.  

Ankle Foot Orthosis (AFO)

Patients may need an ankle foot orthosis to help support their ankle joint, as the muscles surrounding this joint are often affected, making the patient at risk for falls.  Other orthotics to promote hand function and prevent neck pain can be prescribed and fit to the patient.  SLP's focus on improving the patient's ability to chew and swallow, as those muscles tend to be affected, putting the patient at risk for choking and aspirating food and liquids.

Patients with Myotonic Dystrophy also struggle with visual  impairments and upper and lower extremity weakness so often OT's train them in the use of adaptive equipment and mobility aids, such as a long handled reacher and long handled sponge so the patient can independently complete item retrieval,  dressing and bathing.

                       

                   Leg Lifter, Shoehorn, Reacher, Sponge, Sock Aide, Elastic Shoelaces

I met my first patient with Myotonic Dystrophy a few months ago and her particular set of difficulties stuck with me.  She had been diagnosed with Type 1 Myotonic Dystrophy and over the last few years had started to struggle with maintaining her weight, her  mobility and her independence.  Slowly but surely her muscles just weren't responding to her commands; the fingers of her left hand were almost completely rigid in what looked like swan deformities and her right hand was starting to go that way too. 

She already had twenty four hour caregivers who helped her with dressing, bathing, grooming as well as with the more challenging activities of daily living, so after dutifully recommending bathing equipment that would make her more independent (and her refusing to try any of them) I moved on.   I turned my attention to the contractures that were developing in both her hands.  

Resting Hand Splint

Usually what we recommend for neurological conditions that affect the muscles in the hand and wrist is a resting hand splint that will keep the digits and wrist in a neutral position so that over time the contractures don't worsen (but they also aren't likely to improve unless we add a dynamic splint).  

In this case however, I knew the resting hand splint wouldn't be enough to address each digits contractures at the PIP and DIP joints (proximal interphalangeal middle joints of the fingers and distal interphalangeal or tips of the fingers).  So my search continued...

I stumbled across a medieval looking device as I was perusing the contracture management devices on the website of Medical Supplier Sammons and Preston.   During my next visit I proudly showed my patient the picture I had downloaded from the Internet, thinking I had just elevated myself to Super OT.  Instead she said, "Oh the Finger Flexion Glove?" and nodding knowingly.  She then asked her caregiver to go look in her bedroom closet, and lo and behold the caregiver came back with a glove that looked very similar to the one I had found online.   My patient explained that she had used these gloves at least 15 years ago when she was living in Chicago and she had completely forgotten about it.  Her gloves were unfortunately missing a crucial part, the anchor where the elastics are tethered to provide traction on the digits.  After problem solving with the caregiver about various ways we could adapt her existing glove, we found a pair online for $30.24 and my patient ordered them right on the spot.

Finger Flexion Glove

 
 Once the gloves arrived we started my patient on a wearing schedule that she could tolerate (from 10 minutes three times a day working up to 30 minutes, three times a day) and she was so happy to finally have the right tool (again) to help maintain the range of motion and flexibility of her fingers for grasping and manipulating.

So to all the other OT's out there in the Interweb universe, if you are stuck on how to help your patient who has Myotonic Dystrophy, or any other neurological disorder with rigid or contracted fingers in extension, don't forget about the Finger Flexion Glove!

  

[edit]

Graft-Versus-Host Disease and OT

      Its not known how common Graft Versus Host Disease (GVHD) is in the US, but it can occur in 30-40% of patients who received a transplant from a related donor and in 60-80% of patients who received a transplant from an unrelated donor.  Generally when patients are diagnosed with GVHD they are very weak and so receive Physical and Occupational Therapy in the hospital when they are stable enough to participate or once they are discharged to a skilled nursing facility or to their own home.

     There is an acute version of the disease, which usually happens up to three months post transplant, and a chronic version starts after three months post transplant.  Some common acute symptoms include abdominal pain/cramps, diarrhea, fever, jaundice, skin rash, vomiting, weight loss and more chronic symptoms include dry eyes and mouth, hair loss, hepatitis, lung and digestive tract disorders, skin rash and skin thickening.  The treatment for GVHD is still changing as research continues to show the benefit of using different combinations of drugs or steroids but generally it is treated with the administration of Cyclosporine, Methotrexate, Tacrolimus, Sirolimus or Prednisone, which all weaken the donor T-cells enough so they don't attack the patient's organs and tissues.  Unfortunately the side effects of these drugs can cause some very extreme changes in the patients emotional and physical state.  Depression, confusion, anxiety and roller-coaster emotions can make it difficult for the patient and their family or support system.

      It was a Friday afternoon on a sunny Portland day and I was going to meet and evaluate my first GVHD patient.   I had been warned not to come if I was feeling sick, as it was a common cold virus that had sent him to hospital and led to him being diagnosed with GVHD.  I was thinking that most of my focus would be on equipment as the patient had already fallen since being home.  My patient had a history of being an elite athlete and was a successful real estate lawyer so I was guessing he was eager to get back to his life.  Our initial meeting was uneventful; he was talking on the phone and so I spent most of the visit talking with his wife, who showed me the bathroom, updated me on how he was doing and admitted to me that she thought her husband was still in denial.  She explained that he had already told her he didn't want to live like a cancer patient (he had had leukemia before the bone marrow transplant) and that if he couldn't go back to his life it wasn't worth living.

     I must have looked a little shocked at this statement because she explained to me that she had been doing a lot of soul searching on their situation and she thought that this was happening to him for a reason.   She pointed out that the love of his life was his work and that slowing down to spend time with her and their children was something she wanted for him but was afraid it wasn't something he was capable or interested in doing.  As I digested this, I recommended they replace their flimsy towel bars with grab bars, install arms on the toilet and place a vertical grab bar inside the shower to assist the patient with getting in and out more safely.  She wrote this all down and went to get the patient, who grudgingly came into the bathroom.  Knowing that he was likely to resist any changes if they were for him I tried to couch my recommendations in that the equipment would come in handy for when their parents or other elderly people visited them.  He went along with everything I said, nodding as we went, until his spouse pointed out that their parents were too frail to visit anymore, but that this equipment would help him a lot.  This statement challenged the patients belief that he was healed and I knew what was coming.  The patient abruptly refuted everything he had just agreed with and I felt the momentum of the visit slip through my fingers.  Angry at his wife and me, he refused to participate in range of motion, manual muscle testing or other assessments, saying he was too tired and that he had already done all of this with the PT.  I respectfully backed off and offered that I could play a consultant role his his Home Health therapy and they could call with questions or concerns.  We somewhat formally shook hands and I gathered my things to leave.  Although I hope he was happy with this agreement, I know he was even happier to see me leave.

     So it turns out patients with GVHD are just like any other patients.  They are reluctant to believe their body is failing them, they lack insight into their own abilities and they cling to the roles and responsibilities of their previous jobs or hobbies.  All we can do in these tough cases is support the family and caregivers, recommend the equipment that the patient will likely never purchase and wait by the phone in case we are needed.