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Medical Minute 6-1: Viagra Rescues Cold Hands

By: Vanessa Welch Email
By: Vanessa Welch Email

Her costumes have graced the stage of the Chicago Shakespeare theater for 22 years.

"I'm able to be creative about it."

But two years ago, Lise Stec nearly lost her ability to sew.

Days after squeezing the water out of a sponge, Lise's fingertips began to turn black and blue. She'd lived with cold hands syndrome for decades but never anything like this.

"I would say she's one of the most severe cases I've seen in my practice over the last 10 years," said Nadera J. Sweiss, M.D., Co-Director at the Cold Hands Clinic University of Chicago Medical Center.

Raynaud's is common in patients with systemic autoimmune diseases, especially scleroderma.

It limits blood flow to the hands and can lead to discoloration or worse. Lise's case was so severe, she didn't respond to standard treatment or surgery. Ultimately, gangrene set in.

"She had some ulcers at the tips of her fingers, and she had decreased blood flow and probably a month or two after surgery, this is how her hand looked."

"I would look at my hand and go, 'That's not really my hand,' because it didn't, it looked like a gross Halloween hand," said Lise Stec.

Her options were limited: Either amputate one finger or doctors could glue her hand to her buttocks for six months for a skin graft.

"I can't imagine walking around with my hand like that."

Either way, time was running out to save Lise's hand. Without any FDA-approved therapies for severe Raynaud's, a team from the university of Chicago's cold hands clinic came up with an off-label approach: A drug cocktail including Flonan and Viagra to get Lise's blood flowing. Slowly, her hand began improving.

"It is very important to keep the hope and believe in the impossible."

Lise endured a year full of pain and multiple medications. But after a second surgery, she got a new hand.

"So, now, this is all my skin minus my fingertip."

And a chance to get back to what Lise loves.

For more information on other series produced by Ivanhoe Broadcast News contact John Cherry at (407) 691-1500, jcherry@ivanhoe.com.

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MEDICAL BREAKTHROUGHS - RESEARCH SUMMARY:

BACKGROUND: Cold hands syndrome occurs as a result of blocked or constricted blood vessels caused by a vascular disorder (a cold hand disease) or an injury such as: blood clotting, Raynaud’s, hand trauma, tumors or frost bite.
(SOURCE: University of Chicago Medical Center, Cold Hands Clinic)

RAYNAUD'S: In Raynaud's disease, smaller arteries that supply blood to the skin narrow, limiting blood circulation to affected areas. Women are more likely to have Raynaud's disease. It's also more common in people who live in colder climates and among those who have a family history of the disease. Although it can develop in anyone, primary Raynaud's often begins between the ages of 15 and 30.
(SOURCE: Mayo Clinic)

SYMPTOMS: Taking longer than 15-20 minutes for your hands to warm up after they have been exposed to cold temperatures, pain, swelling, numbness, discoloration, wounds that don’t heal, and streaks of discoloration under the fingernails are symptoms of cold hands syndrome.
(SOURCE: University of Chicago Medical Center, Cold Hands Clinic)

TREATMENT: Many patients find relief through medications like Viagra and Cialis to reduce the risk of blood clotting and improve blood flow through the fingers and hands. Antidepressants can help regulate pressure in the blood vessels. Botox injections can relax muscles in the hand.

SURGERY FOR THE WORST CASES: Persistently cold, numb hands are signs of a more severe condition, as in Lise Stec’s case. She was diagnosed with scleroderma and Raynaud’s. When gangrene set in to her fingertips, she underwent digital sympathectomy. Doctors recommended a series of surgeries to remove the dead skin, performed a skin transplant and injected her hand with growth hormones.

FOR MORE INFORMATION, PLEASE CONTACT:
John Easton, Director of Medical Communications
University of Chicago Medical Center
Chicago, IL
John.Easton@uchospitals.edu

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THE FOLLOWING IS AN IN-DEPTH INTERVIEW WITH THE DOCTOR FROM THE STORY ABOVE:

Nadera J. Sweiss, M.D., Co-Director of the Cold Hands Clinic at the University of Chicago Medical Center, discusses cold hands and how she helped in saving her patient’s hand.

Can you discuss cold hands and furthermore delve into some of the symptoms that are associated with it?

Dr. Nadera J. Sweiss: Cold hands is a very common problem. At one point, over our entire lifetime, we will at one point or another suffered from cold hands. It is a normal physiological phenomenon when you are exposed to cold without adequate protection of your hands. However, not every cold hand is a problem, and moreover not every cold hand point to underlying medical problems. If it takes more than 15 – 20 minutes to warm your hands after exposure to the cold, then one would start thinking that perhaps some medical issues are at hand. You can get it at any age. When it is associated with autoimmune diseases, which is my main area of research, it tends to frequently occur in middle-aged men and women. Furthermore, it is generally associated with other features suggestive of an autoimmune disease..

Can you briefly explain the most recent case that you have been working on to aid in the further development of treatment for cold hand?

Dr. Nadera J. Sweiss: This is a young woman with the diagnosis of scleroderma (also known as systemic sclerosis). Scleroderma is an autoimmune disease that is characterized my multiple organ system involvement. Cold hands or Raynaud's is an exceptionally common feature when dealing with this disease. In addition, patients may have lung involvement, gastrointestinal involvement, cardiac involvement and joint involvement. Ultimately, there are different types of this disease. In this particular case, the patient had cold hands that are associated with digital ulcers, as well as significant decrease in blood flow to the hand – she had a lot of dilated blood vessels that we refer to as telangiectasias that were distributed over her chest, face and hands; luckily, she did not have lung or heart involvement. The major features of her disease were the severe cold hands that were very difficult to control, despite adequate hand protection as well as medical treatment.

How long ago did this patient come to you for treatment?

Dr. Nadera J. Sweiss: I have been following her for the last seven years.

So this has been quite a long road then?

Dr. Nadera J. Sweiss: Yes.

Can you discuss how you came about treating this patient?

Dr. Nadera J. Sweiss: She was followed by one of my colleagues. Upon his retirement, I took over the responsibilities of treating her. The main feature of her symptoms at that time were the cold hands. She did not get really sick until 2009, when she noted that the symptoms of cold hand got much worse, and the symptoms were associated with a change in the color of the fingers – the fingers color began to change to black, and they were extremely painful; these particular symptoms were followed by more pain when she began to experience ulcers at the tips of her fingers.

How does her case in particular differ from all of the other patients that you have treated for this specific condition?

Dr. Nadera J. Sweiss: We see many cases like this because we are a referral center, and we get to see some of the more difficult cases. I would say that she had severe Raynaud's syndrome, and I might add that she is one of the more difficult cases that I have encountered over the past 10 years practicing in terms of the intensity of her condition.

What could happen if the symptoms aforementioned (i.e. black to blue fingers) continued to be untreated?

Dr. Nadera J. Sweiss: If left untreated, there is the possibility of developing gangrene in addition to excruciating painful ulcers in the fingers. I will show you some pictures here. This is how Raynaud's syndrome looks. It may affect one digit or more than one digit. It doesn’t always select just one particular finger. Here you see the white phase of Raynaud's syndrome where (as you would think) the fingers turn white. Here you see the blue phase of Raynaud's syndrome. Ultimately, it is not uncommon for patients to develop gangrene at the tips of their fingers with severe untreated raynaud’s. You see how the decrease in blood flow to the little finger in addition to the index finger resulted in gangrene and moreover loss of tissue. This is a different patient that we treated with the same disease, and she failed treatment thus we referred her to a surgery called digital sympathectomy . . . and she did well after surgery. Here is a picture of her hand two years following the surgery; in point of fact, not all patient recover and do as well with surgery as the one previously mentioned and displayed in this photograph. It is imperative to maintain normal blood flow to the hand. That is how we keep our hands healthy. These are the digital arteries that are branches of the major arteries that supply the hand. Each finger has a blood supply, and we call these arteries “digital arteries.” These arteries are surrounded by tiny nerves, and what controls the blood flow is the size of this artery – whether this artery has a minor blood clot in it or not, or if the nerves surrounding these arteries are not functioning in a normal way. So, if you have a spasm in that artery you will have decreased blood flow . If you have a blood clot inside of that artery you will have decreased blood flow. Conclusively, if you have inflammation in the wall of this artery you would decreased blood flow. When we diagnosed this patient, we found that she had severe Raynaud's syndrome – she did not just have scleroderma, which is a disease characterized by decreased blood flow to the digits. She on top of that had antibodies circulating in her blood that we refer to as antiphospholipid antibodies; these antibodies contribute to clotting of the blood vessels. In her case, she had two disorders. She was unfortunate to have on top of decreased blood flow problems with increased blood coagulation, and hypercoagulability in her blood, which further decreased her blood flow. While she developed ulcers in her hands, we tried multiple treatment options to help with her hand. Alas, she did not respond to these treatment options. Additionally, it is important to keep in mind that there are no FDA approved therapies for the treatment of scleroderma and for the treatment of Raynaud's (in her case, a severe case of Raynaud's). So, everything that we do for these patients is essentially off label. Every physician will treat their patient differently, and it boils down to the style in addition to experience of the physician when it comes to treating conditions like this. Furthermore, she underwent a surgery, however, she did not heal well following it because she did not have good flow. The reason that she underwent surgery (despite knowing she did not have good flow) was because none of the other options presented were offering any sort of benefit for her. This is a picture of her hand – with severe pain, you see all of these ulcerations and dead tissue in addition to loss of tissue.

Was it following this picture that she was referred to a surgical team to perform the operation?

Dr. Nadera J. Sweiss: No. – she had some ulcers at the tips of her fingers and she had significant decreased blood flow. After the surgery (approximately a month or two) this is what her hand looked like.

How long ago was her surgery performed?

Dr. Nadera J. Sweiss: It was early 2009.

So about two years or so (give or take a few months)?

Dr. Nadera J. Sweiss: That is correct.

Could she have lost her hand?

Dr. Nadera J. Sweiss: Absolutely. She was at a great risk for losing her hand. She had no blood flow to the hand. When we did the blood flow studies, we did document that blood flow to the hand had significantly decreased despite being on multiple medications and additionally despite going through surgery.

What did you decide to do then following her surgery and seeing the state at which her hand was in?

Dr. Nadera J. Sweiss: I managed this case in collaboration with my colleagues in plastic surgery as well as my colleagues in cardiology. We established a multidisciplinary team to treat this case. There were a multi-fold of options in surgery. One of the options was to glue her hand to the back of her buttocks in an effort to try and get a tissue graft. The success of that was not clear. We are not sure exactly how successful our efforts are going to be at this moment. Furthermore, she declined this procedure. She did not want to go through surgery again .

So, she would have her hand surgically glued to her buttocks for about six months then?

Dr. Nadera J. Sweiss: At least six months. The second option is to cut the fingers, and the problem with cutting the fingers is that if we cut the finger (here or here or here), we know that there is decreased blood flow to this area and we know that this area was inflamed as well and at risk. So our concern was that if we cut these fingers, we may have to cut more and more and more and eventually she may end up losing the entire hand.

So, the options for her at the moment were to either glue her hand to her buttocks in order to obtain a skin graft, where she would have to walk around like this for no less than six months . . . or you would have to ultimately remove her digits and hope that it didn’t spread to the rest of her hand, where you could be forced to remove additional fingers?

Dr. Nadera J. Sweiss: That is correct. That is when she came to me, and her husband came to me, and her son came to me and they said, “Is there any other way that we can save her hand? Is there any other kind of treatment or procedure that we can do to save her hand?”

What did you say to them?

Dr. Nadera J. Sweiss: I further discussed with the family the limited number of trials as well as the lack of funding for research in this area. There was no right or wrong. There was no clear answer. Whatever I was going to offer them was going to be off label. I told that that “. . . if you are going to agree to this, and agree to accept the challenge and the side effects of these medications, and if we get your insurance to pay for some of these medications, then I am willing to work with you and try to avoid surgery. We will perform surgery later on once we increase some blood flow to the hand.”

So then what happened next?

Dr. Nadera J. Sweiss: We admitted her to the hospital. I still remember – it was Thanksgiving 2009. There were many nights that I found myself unable to sleep pondering what the best and most efficient treatments for her would be. I reached out to friends who ran clinics specifically for scleroderma in different parts of the country to see exactly how they would approach such a complex case as this one. They suggested some medications, but I didn’t feel that they were enthusiastic about the outcome in her particular case. When we admitted her to the hospital, we decided to use manifold medications all at the same time. We gave her a medication called flolan, which is used to treat pulmonary artery hypertension. It can be used in some cases of hand ischemia. This is a very costly drug that is administered intravenously. It has many side effects and should be given in a monitored setting. So, we assessed the blood flow to the hand, and then we started the flonan infusion we started Viagra to aid in the blood flow to the hand. We added to this a blood thinner to assist in improving blood flow in case there were blood clots on those tiny blood vessels aforementioned. We used a small dose of prednisone as well in case there was inflammation, and in which case we would be able to control it. We were lucky. After one week we began seeing increased blood flow. Upon seeing these results, we ceased therapy with this flonan IV, however, we continued therapy with the full dose of Viagra in addition to the blood thinner as well as myriad other medications that aided in her recovery . She did well. We did notice as time went by that the blood flow started to improve. That is about the time when first noticed increased blood flow. It is quite a lengthy process.

How long was she taking those medications?

Dr. Nadera J. Sweiss: She took the IV medication for two weeks in the hospital. She stayed on the blood thinning for approximately two months (until we were sure that she was fully healed). She is still taking the Viagra as well as the calcium channel blockers, aspirin in addition to several other medications.

All of these medications were used for the common goal of helping her increase and keep constant blood flow to her hand?

Dr. Nadera J. Sweiss: Yes. When we started seeing improvement in the appearance of the hand, we commenced the next phase of her treatment (plastic surgery), where she went under multiple operations to improve tissue healing and tissue regeneration. It wasn’t only the medical treatment. The only treatment that I did was increasing the blood flow so that the surgeons could perform their operations and allow her to once again return to a healthy and normal quality of life. We knew that she would do well, but sure enough, it took some time and ended up being quite a lengthy and tough journey – I had to prescribe her with morphine for more than a year just to help control the horrible pain that was associated with the condition as well as numerous operations. But as you can see the hand started healing. This is a recent picture of her hand. Yes, there is some difficulty for her with making a full grip; her hand function is not 100 percent, but it is far better than losing her hand. She is still able to use her hand and perform all of her daily activities without too much inconvenience.

So, she just lost the tip of her ring finger?

Dr. Nadera J. Sweiss: That is correct.

You know, really, that is nothing. She could have lost her whole hand.

Dr. Nadera J. Sweiss: Absolutely.

All of this progress has come within the last two years; am I correct in saying that?

Dr. Nadera J. Sweiss: That is correct.

What is it like for you as a doctor to see her going from the first pictures that you showed me where it looked as if her flesh was falling from her hand (she had the risk of ultimately losing it), to the point that she is at now?

Dr. Nadera J. Sweiss: I am humbled and honored to help with these cases everyday in my practice. It keeps me on my toes. It teaches me that I have to be a student at all times in my life no matter how experienced we consider ourselves to be. There is a lot to learn. There is a lot to do. It is very important to always keep that hope and faith in the impossible.

You mentioned before that when you approached some of the other physicians regarding her case, they seemed almost pessimistic and almost expected the worst, is that correct?

Dr. Nadera J. Sweiss: Yes. The patient did not want to lose her hand. After all, as a physician I believe that we must always put the patient and their family’s first, and work with them closely to determine what they want to be done with their bodies. Patients should be partners in their health care. As physicians, we try to follow the sciences. I am a clinical trialist; I believe in randomized clinical trials – but good luck with diseases like this. We will never be able to do the randomized clinical trials that we dream about. The fact that there are no FDA approved therapies for such a disease, it will not prevent me from doing all that I can to help in cases like this one. Based on the evidence that we have acquired from this case and other cases like this one, as long as our patients are aware of the risks that we are taking, and as long as they are willing to take that risk, and as long as we are experienced in using these medications, and moreover know how to work around the side effects associated with these medications, we will continue to aid in their treatment in recovery as best as we possibly can.


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