Scleroderma with renal failure

1. Scleroderma with renal failure Presented by Tandis Bishop

2. Case Summary 62 yr old women admitted to hospital #2 due to renal failure. 6 weeks before admission pt noted: • skin tightness around her mouth • dry mouth • difficulty swallowing • worsening gastroesophageal reflux • hair loss, discoloration of her fingers (ash-colored), painful and cool. 4 days before admission – found on floor, confused, not fully cognitive, weak

3. Systemic sclerosis or Scleroderma What is it? • connective tissue disease - decomposition of fibrous connective tissue in the skin, blood vessels, muscles, and internal organs. • autoimmune disorder- immune system mistakenly attacks and destroys healthy body tissue. • buildup of collagen in the skin and other organs - leads to the symptoms of the disease. 2 Main Types: Limited (CREST syndrome) and Diffuse Types of Scleroderma. John Hopkins Scleroderma Center. 2012. http://www.hopkinsscleroderma.org/scleroderma/types-scleroderma/. Accessed April 8, 2012.

4. Cont. • 30 to 50 yrs old • More common in females than males • can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis Associated with: Sclerodactyly(thickening/tightening skin of fingers/toes) Raynaud’s phenomena (coldness of small extremities) Telangiectasia (widened blood vessels on skin) Xerostomia Sjogren syndrome (no tears/saliva) Dysphagia 70% of diffuse scleroderma – renal involvement Scleroderma. PubmedHealth. 2012 http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/. Accessed April 8, 2012.

5. Signs & Symptoms A.D.A.M. Medical Encyclopedia. Pubmed Health (2012)

6. S/S cont. • Skin hardness, thickening, stiffness, and tightness • Hair loss • Sores (ulcers) on the fingertips or toes • Joint pain, wrist pain, numbness and pain in the feet • Breathing problems (scarring of lungs) Other GI problems may include: • Gastroesophagealreflux • N/V • Diarrhea • Bloating after meals • Constipation • Malabsorption of lactose, vitamins, minerals, and fatty acids • Problems controlling stools (fecal incontinence) What is Scleroderma. Scleroderma Foundation. 2012 http://www.scleroderma.org/medical/overview.shtm. Accessed April 12, 2012.

7. Permission to use for class presentation

8. Diagnosis/Test Physical exam- show hard, tight, thick skin. Blood tests: • Antinuclear antibody (ANA) panel • Antibody testing • ESR (sed rate) • Rheumatoid factor Other tests: • Chest x-ray • CT scan of the lungs • Echocardiogram • Tests to assess lungs and GI function • Skin biopsy Scleroderma. University of Maryland Medical Center. 2012 http://www.umm.edu/altmed/articles/scleroderma-000147.htmAccessed April 12, 2012.

9. Treatment No Cure Control symptoms and prevent complications: • Corticosteroids • Immunosuppressants • Nonsteroidalanti-inflammatory drugs (NSAIDs) Other txfor specific symptoms may include: • Meds for GERD or dysphagia • ACE inhibitors for BP control and Kidney problems • Relieve skin thickening • Breathing • Treat Raynaud's phenomenon • Physical therapy Scleroderma. University of Maryland Medical Center. 2012 http://www.umm.edu/altmed/articles/scleroderma-000147.htmAccessed April 12, 2012.

10. Anthropometrics • Wt 55 kg, Ht 163 cm • BMI 21 kg/m2 (normal) • UBW 58 kg • Unintentional wt loss 3kg in 6 wks, • 5.2% wt loss (significant)

11. Biochemical

12. Clinical Pt. had an polymyositis evolved into a diffuse cutaneous scleroderma-polymyositis overlap syndrome with acute renal failure, hypertension, encephalopathy, and possibly a seizure.

13. Clinical - PMHx • Hypertension • Osteoporosis • Depression • Multinodular nontoxic goiter Family Hx Sister – Systemic lupus erythematosus – died at age 27

14. Clinical - HPI 26 mos before admission • Bone marrow-biopsy • Dx:monoclonal gammopathy of undetermined significance (MGUS) – presence of abnormal M protein in blood made from WBC 17 mos before admission • Increasing limb weakness and head drop • Dx: autoimmune overlap syndrome with polymyosistis • Prednisone and mycophenolatemofetil

15. Clinical - HPI 7 mos before admission • Persistent, severe weakness and atrophy in the neck muscles • Electromyogram (EMG): active myopathy 5 mos before admission • Mycophenolatemofetil 2 wks before admission - Fell at home, injured back – naproxen (NSAID) daily for pain

16. Hospital #1 – Day 1 CT scan • small hemorrhagic infarct GFR – 6 Admitted to ICU Meds: IV, furosemide, chlorothiazide, methylprednisolone, nicardipine drip, ceftriaxone, atenolol, nitroglycerine paste, mycophenolatemofetil, and famotidine.

17. Hospital #1- Day 2 • MRI – acute infarction • Renal ultrasound - ↑ echogenicity • ECG - ↑ heart stress • EEG – abnormalities consistent with toxic metabolic encephalopathy (brain disease) • Day 4 transferred to hospital #2

18. Diagnostic Labs

19. Hospital #2 Day 1 • Exam showed marked thickening and puckering of skin around mouth, dry oral mucosa, supple neck and muscle wasting • Low urine output, pro in urine • IV, furosemide Day 2 • Oliguria persisted Day 3 • Renal biopsy • Dx – Scleroderma renal crisis

20. Diet Hx24-hr Recall Breakfast – ¾ cup cold cereal ½ cup of 2% milk Lunch – 1pre-made frozen beef and bean burrito (microwaved) 1 ½ cup iced tea, sweetened Dinner – 1 cup spaghetti with ½ cup marinara sauce 900 Kcal, 20g Pro

21. Energy, Protein, Fluid • Energy: 1700 kcal (30 kcal/kg/d) • Protein: 40g (0.75g/kg/d for ESRD) • Fluid: 1700 ml (1ml/kcal)

22. Diagnosis Primary: Inadequate oral intake food/beverage (NI 2.1) R/T swallowing and eating difficulties secondary to scleroderma AEB significant wt loss of 5.2% in the past 6 wks and 24-hr recall. Alternate: Involuntary wt loss (NC 3.2) R/T inability to consume adequate energy AEB significant wtloss of 5.2% in the past 6 wks and pt only meeting 53% of energy needs.

23. Goal Increase patient’s caloric intake by 800 kcals Increase patient’s protein intake by 20g Prevent further weight loss

24. Intervention • Begin NG tube feeding of 1.5 kcal enteral formula at 25cc/hr. • Increase 10 cc every 4 hrs to goal rate of 55cc/hr with 30ml flush q 4 hrs. • Transition to soft, mechanical oral diet as conditions improve

25. Intervention • Consume soft, energy-dense foods such as mashed potatoes with gravy, scrambled eggs, mayonnaise, creamy soups, cooked cereals (oatmeal or cream of wheat) with cream, milkshakes • Consume high protein foods such as ground meats, tuna, cottage cheese, baked beans, soft tofu • Consume 2 cans of high-protein Boost between meals (1X can before L, 1X can before D) • Eat small, frequent meals, slowly, chewing well • Moisten dry foods with sauces, broth, dressing, or gravy. • Drink plenty of fluids, continuously sipping throughout the day • Take a daily multivitamin/mineral supplement

26. Communication • Provide information on ways to increase intake with sx like dysphagia, dry mouth, and other feeding difficulties. • Discuss the effects of medication on diet & appetite. • Discuss with social worker for possible long-term care needs and placement after hospital release

27. Handout Eating Well: Nutritional Needs in Scleroderma By LiseGloede, Registered Dietician (originally published in "Scleroderma Voice," 2001, #4) The purpose of this article is to identify nutritional needs in scleroderma and provide practical food advice, with the two goals of minimizing nutrition-related side effects and to improve your overall nutritional health. Since scleroderma can be manifested in different ways and each person may have differing nutritional needs, there is no one "diet for scleroderma." This article will discuss nutritional therapy for mouth, esophageal, and swallowing problems, calcium and fiber intake, and alternate ways of nutritional intake. There are many nutrition-related side effects in other parts of the body, such as kidney problems, that require nutritional intervention and modifications to what you eat. However, due to space limitations, not all of these will be discussed in this article. If you want to address your individual nutritional needs related to scleroderma in more detail, you should contact a Registered Dietitian and set up a consultation. Step 1: Food Preparation and Intake • The eating process starts with holding the food to prepare it, then gripping the food to get it into your mouth, and then follows the chewing and swallowing process. • This is not always easy, especially if scleroderma has hardened the skin on your fingers and hands. If this problem hinders your food intake: • Get kitchen equipment and utensils with thick rubber handles to aid in gripping. • Try various types of cups and mugs until the size and handle configuration are the easiest to pick up. • Stock up on frozen meals or prepared food when possible. • Buy precut fruits and vegetables to help if your joints are stiff and you are having trouble cutting. http://www.scleroderma.org/medical/gastro_articles/gloede_2001.shtm

28. Cont. Step 2: Your Mouth • The easiest way to discuss nutrition therapy for various body areas is to go through the digestive process and follow the food on its course, discussing the problems that may occur along the way. • The mouth is the first place the food goes, and there it is mixed with saliva. However, if dry mouth occurs, as is common with Sjogren's syndrome or as a result of taking some types of antidepressants, take the following steps: • Drink plenty of fluids, continuously sipping throughout the entire day. • Take sips from a water bottle (or plastic squirt bottle, which can be helpful if lip and mouth movements are difficult). • If you can, chew sugar-free gum or pop a sugar-free candy into your mouth to stimulate saliva production. • Use artificial saliva (for example, "Salivert," "Optimoist," or "Salix Lozenges"), available at drugstores. Step 3: Swallowing (Mouth through Esophagus) • Overproduction of collagen due to scleroderma can cause thickening and scarring of tissue. Weakened muscles can cause slow movement of food which is called "dysmotility." Difficulty in swallowing is called "dysphagia." This can also result from narrowing of the esophagus. When these problems occur: • Eat slowly. Allow more time for eating, due to slower movement of food passing through. • Chew well. Be careful not to take any food into your lungs (aspiration) • Eat soft or pureed foods (mashed potatoes, applesauce). Moisten dry foods like bread or crackers by dipping them into liquids. • Drink water or other fluids frequently, and between bites to help the food go down. • Blenderize foods, especially meats or vegetables. Add seasonings, broth, or margarine to thin to desired consistency. http://www.scleroderma.org/medical/gastro_articles/gloede_2001.shtm

29. Monitor / Evaluation • F/U weekly, dispatched F/U 1X month • Weightwith each visit • BP • Labs • Glucose, Lipids, Electrolytes, BUN/Creatinine • Monitor tolerance to tube feeding • Monitor presence and/or improvement symptoms • Monitor oral intake ( 3-day food record) • F/U with pt on toleration of oral diet

30. PICO Question In healthy adults, does eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) supplementation versus no supplementation, enhance immune function? P: Healthy adults I: EPA and DHA supplementation C: No supplementation O: Enhance immune function