April/May 2010 Issue
Though best known for her roles in the hit Scary Movie series and on television’s Ally McBeal, some of Regina Hall’s proudest work takes place well away from the glare of Hollywood. An avid volunteer for the care of senior citizens and an advocate for more public awareness of scleroderma, Hall, along with her mother Valery, sat down with ABILITY’s Chet Cooper to discuss the rare skin condition and how it has hit her family close to home.
Chet Cooper: Regina, when we were talking earlier, you’d mentioned that your mom had been diagnosed with scleroderma. Can you tell me a little bit about that experience for the two of you?
Regina Hall: My mom was diagnosed with scleroderma about six years ago. It affects the skin and some other organs, and can take several forms. The type my mom has is called CREST. Fortunately she has an amazing doctor at Johns Hopkins. I’ve been learning a lot about the condition, and I know that it predominantly affects women. There’s not a lot of funding for research and treatment of scleroderma, unfortunately.
Cooper: It’s fairly rare, isn’t it?
Regina: It is. But you know, it’s still more common than people think. In the early stages of the condition, it looks like lupus. It can cause a hardness of the skin, blotching, development of red spots. In the most severe cases, you develop acid reflux because your esophagus begins to harden and swell, so breathing eventually becomes difficult. Fortunately, my mother’s case isn’t that intense, so that’s a blessing. But she does have Raynaud’s, so when she’s cold, she loses circulation in her hands and feet. They turn white. So she doesn’t really do grocery stores for long periods of time anymore.
Cooper: Because it’s difficult to stand or walk for long periods of time?
Regina: No, because she’s affected by colder temperatures at the grocery store. It’s really important for her to keep her hands and feet warm. Even when she’s just out driving, she wears warm mittens or driving gloves. She lives in Washington, D.C. but tries not to spend too much time outside when it’s cold.
Cooper: So people that are in parts of the country that are colder would have more of a-
Regina: Certainly when you drive, you have to put on warm mittens. She lives in Washington DC. She wears driving gloves and she tries not to spend too much time where the temperatures are cold outside.
Cooper: Valery, I know you’re technically retired, but also that you still do some substitute teaching out in D.C. How is that experience? There are some tough neighborhoods out there.
Valery Hall: Yeah, but I was born in one of the tough neighborhoods. So when I was teaching, I came into my elementary schools as if they were any other elementary schools. I knew all the kids and they loved me, I loved them. All my kids finished high school. Didn’t anybody get killed or anything like that.
Cooper: How is your health otherwise? The two of you had mentioned something about a stroke earlier.
Regina: Yes. My father died in 1994 of a massive stroke. My mother had a mild stroke in February, but she’s been doing wonderfully. She does rehab three days a week and it’s really been great, too, helping her strengthen her left side. She’s been stair-climbing, she’s been walking. So it’s been good.
Valery: Yeah, I feel fine. But when I first had my stroke, it was the night of a big blizzard in Washington. The medics couldn’t even drive up to the house, they had to walk up. The nurse told me my pressure was 120 over 150 and that I didn’t have to go to the hospital. So I didn’t go, but that Thursday morning I woke up and I was feeling so bad. By Friday morning I couldn’t hardly make it to the kitchen to get any food. So I decided maybe I should go to the hospital.
Regina: I read an amazing book for anyone whose family member has suffered a stroke. It’s called My Stroke of Insight, and is about a woman, a brain scientist, who had a stroke on her left side and so only her right side was undamaged. It’s a really great book for understanding strokes and what happens.
Cooper: I think I’ve talked to her.
Regina: She wrote that she was extremely cognizant of the whole experience, even though her language center wasn’t able to recall language. She still knew what was going on. And the fact that she was using herself as a case study is the best part. Pretty amazing.
Cooper: Kirk Douglas wrote a book, called My Stroke of Luck, about how he looked at life and therapy after his stroke. At 83, he realized life was about helping others, about giving back.
Regina: Just last year, I started volunteering at a convalescent home in Sherman Oaks, twice a week. A lot of those patients, the seniors, had had strokes. I guess I had just assumed that these people wouldn’t or couldn’t understand me. But I would go through trivia and current events with them. Some of the people would answer my questions even when I wasn’t sure they’d be able to talk at all. And I’d realize, “Oh, my gosh, they hear! They’re totally cognizant.” It’s amazing how the brain works to survive and recreate.
Valery: My therapy and my diet have really been helping. My daughter has me on a very strict diet. Today was the first day I had coffee. She’s got me on a lot of carrot juice. It doesn’t have the best taste, but I swallow it.
Regina: When my mom was diagnosed, I didn’t know much about the condition. But Dana Delaney, who is a friend of mine, put me in touch with Bob Saget. Bob had made a television movie about scleroderma years ago because his sister had died from it. That was back when they didn’t even know what it was. Anyway, Bob had a group called the Scleroderma Foundation, so I donated to that and my mother went to the doctor Bob had suggested, who happened to be over at Johns Hopkins. He’s been great.
Truthfully, most people only hear about the kind of scleroderma that hardens your organs or turns your skin to stone until you just die. So when my mother was diagnosed, it just sounded like a death sentence. But there are treatments that make it better. It doesn’t have to be a death sentence.
Valery: It was scary, though.
Hall: Yeah, and the thing is, I think the biggest hurdle in the beginning was that she was really not herself. Her energy was completely gone, she was really tired, always sleeping. Now she’s up all day. I can’t stop her! She’s running upstairs now! [laughter] Her energy is really coming back. She’s showing off. I have to make sure she’s not hanging out at the club or roller skating!
Cooper: You’ve got a new movie coming up, Death at a Funeral. Was that fun to work on?
Regina: It was. I’m excited for it. That comes out April 16th. It’s a great ensemble, and is actually a remake of a very funny British film that Frank Oz directed.
Cooper: And you’re still volunteering?
Regina: Yeah, I still work with seniors about two days a week. I think there should be a lot of respect paid to the lives they’ve lived and the lessons they’ve learned. I learn something from them all of the time. One of the nicest things about it is that the people I help don’t know I’m an actress at all. They just have a lot of joy that I come and spend time with them. There’s an all-purpose room where we all do games and trivia. They’re pretty great. They love it when I bring my dog in, too.
Cooper: There’s not a problem bringing uncertified dogs in?
Regina: Well, they just have to make sure the dog has had his shots, which mine has. There’s one woman in my classes who is blind, doesn’t speak any English, loves my dog. And it’s great for me, too, because it means I don’t have to give him so much attention when I come home.
Cooper: Do you do any other kinds of volunteering?
Regina Hall standing in front of Kristian Olson original piece of art.
Regina: I mostly work with seniors, but two weeks ago I helped with The Make-a-Wish Foundation, which is all for kids. I was in a booth where the kids come up and throw a ball and knock down little characters. It was fun.Cooper: How long have you been connected with Bob Saget?
Regina: I met Bob about six years ago. He actually called me once I’d spoken to Dana. He called me about my mother, and ever since then he’s kept me abreast of all of their goings-on.
Cooper: Do you work with them?
Regina: Sure. Every year, they have a big fundraiser, and the biggest thing for them is raising awareness of the disease. A lot of people have still not heard of scleroderma. It’s really about raising the money to continue research. As of now, there is no cure, so all they can do is find ways to treat it and slow its progression. Doctors don’t know what causes it, so they’re still trying to figure out whether it’s something external or genetic.
Scleroderma is a chronic autoimmune disease characterized by fibrosis (or hardening), vascular alterations, and autoantibodies. There are two major forms: Limited cutaneous scleroderma (or morphea) mainly affects the hands, arms and face, although pulmonary hypertension is frequent. Diffuse cutaneous scleroderma (or systemic sclerosis) is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and lungs, and can be fatal. There are no treatments for scleroderma itself, but individual organ system complications are treated.
Prognosis is good for limited cutaneous scleroderma. Prognosis is worse for diffuse cutaneous disease, particularly in older age, and for males. Death occurs most often from pulmonary, heart and kidney complications. In diffuse cutaneous disease, 5-year survival is 70%, 10-year survival 55%.
The cause is unknown. Scleroderma runs in families, but the genes have not been identified. It affects the small blood vessels known as arterioles, in all organs. First, the endothelial cells of the arteriole die off, along with smooth muscle cells, by a process of apoptosis. They are replaced by collagen and other fibrous material. Inflammatory cells, particularly CD4+ helper T cells, infiltrate the arteriole, and cause further damage. Many of the inflammatory and destructive protein signals have been identified, and they are potential targets for drugs that could interrupt the process.
Scleroderma is characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile, and which give the appearance of hidebound skin, a disease occurring in both localized and systemic forms:
This disease is found among all races worldwide, but women are four times more likely to develop scleroderma than men. In the United States, approximately one person in 1,000 is affected. Children rarely suffer the systemic type, but localized scleroderma is common. Most adults are diagnosed after their 30th birthday and before age 50. The disease has high rates among the native American Choctaw tribe and African-American females.
Typical scleroderma is classically defined as symmetrical skin thickening, with about 90% of cases also presenting with Raynaud’s phenomenon, nail-fold capillary changes, and anti-nuclear antibodies. Patients may or may not experience systemic organ involvement. Atypical scleroderma may show any variation of these changes without skin changes or with finger swelling only. Additional symptoms of scleroderma typically present themselves within two years of Raynaud’s phenomenon.
Laboratory testing can show anti-topoisomerase antibodies (causing a diffuse systemic form), or anti-centromere antibodies (causing a limited systemic form, and the CREST syndrome). Other autoantibodies can be seen, such as anti-U3 or anti-RNA polymerase.
Severe complications from Scleroderma include:
• Heart – Untreated high blood pressure strains the heart, irregular heart rhythm and enlargement of the heart lead to heart failure.
• Kidney – Blood vessel damage causes blood pressure to rise, which untreated, will result in brain swelling, headaches, retinal damage and seizures.
• Lung – Two-thirds of all patients suffer from respiratory problems such as shortness of breath, coughing, difficulty breathing, alveolitis (inflammation of lung air sacs), pneumonia and cancer.
• Digestive – Esophagus damage can make it difficult to swallow food, and acid reflux is common. A sluggish intestine may cause pain & bloating; undigested food can result in diarrhea, weight loss and anemia.
• Skin & Joints – Carpal tunnel syndrome is common, as is muscle weakness, joint pain & stiffness.
There is no direct cure for scleroderma. Because the exact cause is unknown, any treatment is patient-specific and aimed at ameliorating symptoms of the disease. For example, patients who experience Raynaud’s phenomenon may be treated with agents to increase blood flow to the fingers, including nifedipine, amlodipine, diltiazem, felodipine, or nicardipine.
Fibrosis of the skin has been treated with varying degrees of success with agents such as d-penicillamine, colchicine, PUVA, Relaxin, and cyclosporine.
Because scleroderma is an autoimmune disease, one of the major pillars of treatment involves the use of immunosuppressive agents. These drugs include methotrexate, cyclophosphamide, azathioprine, and mycophenolate.
Individuals with morphea or limited scleroderma have a relatively positive outlook. They will usually succumb to another disease, not the scleroderma. Those with very widespread skin and organ involvement (systemic) have a negative prognosis. More women have scleroderma, but the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient’s age at diagnosis, the more likely they are to die from the disease.
More information about scleroderma: srfcure.org